9

Uveitis and Ocular Inflammation: Updated Index

2019–2020 BCSC Basic and Clinical Science Course™

Editorial Committee H. Nida Sen, MD, MHSc, Chair Thomas A. Albini, MD Bryn M. Burkholder, MD Sam S. Dahr, MD, MS Emilio M. Dodds, MD Thellea K. Leveque, MD, MPH Wendy M. Smith, MD Daniel V. Vasconcelos-Santos, MD, PhD Note: This index updates pp. 361–383 of Section 9 and is also available online at www.aao.org/bcscindex_s09. Index

(f = figure; t = table) Abatacept, for uveitis, 112 Adaptive immune response/adaptive immunity Accessory molecules, helper T cell expression of, 31 conjunctiva and, 53 Acquired immunodeficiency syndrome. See AIDS definition of, 1 Acrodermatitis chronica atrophicans, 228 effector responses of Actinomycetes, infectious scleritis caused by, 119f antibody-­dependent cellular cytotoxicity, 47–48 Activated neutrophils, 10 antibody-­mediated, 39f, 39–42, 40t Acute anterior uveitis. See Anterior uveitis, acute lymphocyte-­mediated, 42–46, 43f, 44t Acute macular neuroretinopathy, multiple evanescent natu­ral killer cells in, 46 white dot syndrome and, 189 types of, 38t Acute posterior multifocal placoid pigment innate immune response versus, 27 epitheliopathy (APMPPE) mechanisms of, 36, 36f, 38 ce­re­bral vasculitis and, 170 purpose of, 27–28 characteristics of, 162–164t subclinical presence of, 6 definition of, 169 Adaptive T regulatory cells, 34 diagnosis of, 170–172, 171–172f Adeno-­associated virus, 58 fluorescein angiography findings in, 163t, 170, Adenovirus, 272 171f Adult T-­cell leukemia/lymphoma, 265, 266f fundus autofluorescence findings in, 163t, 171, Aedes aegypti, 267 172f Afferent lymphatic channels. See Lymphatics indocyanine green angiography findings in, 163t, Afferent phase, of immune response arc, 29f, 29–30 170–171, 171f AIDS. See also HIV manifestations of, 170, 170f bacillary angiomatosis of, 240 medical management of, 93 bacterial keratitis in, 334 optical coherence tomography findings in, 164t, CD4+ T cells in, 327 171, 172f Cryptococcus neoformans choroiditis in, 333 posterior uveitis in, 169–174, 170–173f cytomegalovirus retinitis in, 256, 328–329 prognosis for, 172–173 external eye manifestations of, 333–334 relentless placoid chorioretinitis, 173f, 173–174 herpes zoster and, 334 ret­i­nal pigment epithelium involvement by, 171 immune recovery uveitis in, 329 serpiginous choroiditis versus, 171 Kaposi sarcoma associated with, 333, 333f systemic conditions associated with, 169–170 molluscum contagiosum associated with, 334 vision loss risk ­factors in, 172–173 necrotizing herpetic retinitis in, 330, 330f Acute ret­i­nal necrosis, 250, 251–252f, 324, 330 ocular toxoplasmosis in, 281–282, 282f Acute ret­i­nal pigment epitheliitis (ARPE), 162–164t, ophthalmic manifestations of, 327–333, 328f, 189f, 189–190 330f, 332f Acute syphilitic retinitis, 222, 224f opportunistic infections in, 328 Acute zonal occult outer retinopathy (AZOOR) pathophysiology of, 327 characteristics of, 162–164t Pneumocystis jirovecii choroiditis in, 332, 332f course of, 191–192 toxoplasmic retinochoroiditis in, 330f, 330–331 definition of, 190 Albendazole differential diagnosis of, 191–192 for diffuse unilateral subacute neuroretinitis, 288 fluorescein angiography findings in, 163t, 191 for visceral toxocariasis, 284 fundus autofluorescence findings in, 163t, 191, 192f Alkylating agents optical coherence tomography findings in, 164t, for Behçet disease, 217–218 191, 191f for uveitis, 109–110 ret­i­nal pigment epithelium findings in, 190, 190f Allelic diversity, in ­human leukocyte antigens, 63 visual acuity in, 191 Allergic conjunctivitis, 2 white dot syndromes versus, 192 Allergic immune reactions, 2 Acyclovir Alpha chemokines, 23t for herpes simplex virus, 249 Altered self, 30 for varicella-­zoster virus, 252 Alternative pathway activation, of complement, 17, 18f Adalimumab Alveolar macrophages, 3 for Behçet disease, 216, 218 American Uveitis Society, 250, 250t for uveitis Amoxicillin, for Lyme disease, 231t description of, 110–111 Amphotericin B, for Candida fungal postoperative intermediate uveitis, 150 endophthalmitis, 298–299 sarcoidosis-­associated uveitis, 199 Anaphylatoxins, 18 treatment studies of, 100–101t Ancylostoma caninum, 287 Angle-­closure glaucoma, secondary, 318 Busacca nodules in, 78, 80f Ankylosing spondylitis (AS) characteristics of, 70 acute anterior uveitis in, 130f, 131–132 chronic ­human leukocyte antigen haplotype association diagnostic criteria for, 141 with, 64 in juvenile idiopathic arthritis, 141–145 laboratory tests for, 125t in pseudophakic bullous keratopathy, 138f nonsteroidal anti-­inflammatory drugs for, 132 cytomegalovirus-­related, 248–249, 257 treatment of, 132 differential diagnosis of, 129 Anterior chamber epidemiology of, 129 immunization in, 56 evaluation of, 71–72t immunologic microenvironment of, 52t, 55–56 fibrin in, 19 immunoregulatory systems of, 55–57 granulomatous, 277 shallowing of, 318 HLA-­B27–­associated Anterior chamber paracentesis ankylosing spondylitis, 130f, 131–132 in bacterial endogenous endophthalmitis, 295 clinical pre­sen­ta­tion of, 130, 130f technique for, 92 description of, 64, 65t in uveitis, 90–92 inflammatory bowel syndrome, 133 Anterior chamber-­associated immune deviation pattern of, 130 (ACAID), 56–57, 59 psoriatic arthritis, 133, 134f Anterior scleritis reactive arthritis syndrome, 132–133, 133f diffuse, 116, 118 incidence of, 129 nodular, 116, 118–119, 119f inflammatory cells in, 77, 79f subtype of, 116t interstitial keratitis and, 220 Anterior segment intraocular pressure in, 80–81, 317 Lyme disease-­related inflammation of, corticosteroids iris nodules in, 77–78, 80, 80f for, 230 keratic precipitates in, 77–78, 79f sympathetic ophthalmia findings in, 200 posterior synechiae in, 77, 80f syphilis findings in, 221–222 prevalence of, 68 ultrasound biomicroscopy of, 89 in sarcoidosis, 195–196 Anterior synechiae, 318 signs of, 77–81, 79–80f Anterior uvea sudden-­onset, 71t, 76 B cells in, 56 symptoms of, 76, 77t immunologic microenvironment of, 52t undifferentiated, 146–147 metastasis to, 309 Antibodies. See also Immunoglobulin(s) T cells in, 56 anti-­idiotypic, 41 Anterior uveitis aqueous humor production of, 91 acute in chronic inflammation, 42 in ankylosing spondylitis, 130f, 131–132 classes of, 39 antimetabolites for, 130 domains of, 39 characteristics of, 64, 65t, 79f, 130–131 Fab region of, 39 chorioret­i­nal scars with, 135f Fc portion of, 39 corticosteroids for, 130 hypervariable region of, 39 cycloplegics for, 131 idiotopes of, 41 drug-­induced, 139–140f, 139–141 local production of, 41–42 fibrinous exudate in, 130, 130f monoclonal in glaucomatocyclitic crisis, 135–136 description of, 41 herpes simplex virus as cause of, 247–250 recombinant, 41 HLA-­B27 diseases associated with. See Anterior plasma cell production of, 37 uveitis, HLA-­B27–­associated structural features of, 39, 39f, 40t hypopyon in, 130, 131f, 214f Antibody Fc receptors, 12 in infectious endophthalmitis, 137 Antibody-­dependent cellular cytotoxicity (ADCC), in inflammatory bowel disease, 133 47–48 intraocular lens-­associated, 137–139, 138f Antifungals lens-­associated uveitis, 136f, 136–137 for Candida fungal postoperative endophthalmitis, in psoriatic arthritis, 133, 134f 298 in reactive arthritis syndrome, 132–133 for chronic fungal postoperative endophthalmitis, seronegative spondyloarthropathies associated 293 with, 131–134, 133–134f Antigen in tubulointerstitial nephritis and uveitis definition of, 27 syndrome, 134–135, 135f immune privilege with, 56 tumor necrosis factor-­ inhibitors for, 131 immune pro­cessing of, 30, 33f aqueous flare in, 77, 79f Antigenic epitopes, 27, 41 in Behçet disease, 214 Antigenic epitopic fragments, 30 Antigen-­presenting cells (APCs) in birdshot chorioretinopathy, 163t, 166, 167f class I-­dependent, 31f in multifocal choroiditis and panuveitis syndrome, class II-­dependent, 30, 32f 163t, 179, 180f 186–187f in conjunctiva, 53 in multiple evanescent white dot syndrome, 163t, corneal lack of, 54 186f definition of, 29–30 in punctate inner choroiditis, 163t ­human leukocyte antigen type and, 63 in serpiginous choroiditis, 163t, 176f in immune response arc, 29–30 in subret­i­nal fibrosis and uveitis syndrome, 163t interleukin-12 production by, 35 in uveitis evaluations, 89 macrophages as, 14 Autoimmune regulator–­deficient mice, 62 monocytes as, 3 Autoimmune retinopathy, 192–193 in phagocytosis, 30, 32f Azathioprine Antiglaucoma medi­cations, anterior uveitis caused for Behçet disease, 217 by, 141 for uveitis, 106–107 Anti-­idiotypic antibodies, 41 AZOOR complex of diseases, 192 Antimetabolites for acute anterior uveitis, 130 B cells for uveitis, 106–108 activation of, 35 Antinuclear antibody testing in anterior uvea, 56 in granulomatosis with polyangiitis, 159 antigen receptors of, 35 in systemic lupus erythematosus, 156 clonal expansion of, 37 Antiretroviral therapy, for cytomegalovirus retinitis, clonality of, 41, 306 256 Fab fragment of, 41 Anti-­vascular endothelial growth ­factor, 274 homing of, 38 Apoptosis infiltration of, 41–42 cytotoxic T cell inducement of, 46 in multifocal choroiditis and panuveitis syndrome, Fas ligand inducement of, 57 179 Aqueous drainage, for uveitic glaucoma, 320 in posterior segment, 57 Aqueous flare, in anterior uveitis, 77, 79f priming of, 30 Aqueous fluid polymerase chain reaction testing, in in resting state, 37 uveitis evaluations, 90–91 tissue infiltration of, 41–42 Aqueous humor tuberculosis response by, 28 antibody production in, 91 B lymphocytes. See B cells biological ­factors in, 55 Babesiosis, 230 polymerase chain reaction testing of, in uveitis Bacillus spp evaluations, 90–91 B cereus, hemolysin BL toxin derived from, 9 Aqueous suppressants, for uveitic glaucoma, 319 endogenous endophthalmitis caused by, 294 Arachidonic acid (AA), eicosanoids from, 19 Bacterial endophthalmitis, endogenous, 294–295 Argyll Robertson pupil, 223 Bacterial uveitis Arrestin, in experimental autoimmune uveoretinitis, 61 bartonellosis as cause of. See Bartonellosis Arthritis leptospirosis as cause of, 232–233 enthesitis-­related, 142 Lyme disease as cause of. See Lyme disease juvenile idiopathic. See Juvenile idiopathic arthritis nocardiosis as cause of, 233 psoriatic polymerase chain reaction for, 90 acute anterior uveitis in, 133 syphilis as cause of. See Syphilis clinical pre­sen­ta­tion of, 133, 134f tuberculosis as cause of, 234–240, 235–238f. See also laboratory tests for, 125t Tuberculosis reactive Whipple disease as cause of, 243–245, 244f acute anterior uveitis in, 132–133 Band keratopathy diagnostic criteria for, 132–133, 133f calcific, 143f, 313 ­human leukocyte antigen association with, 65t, in chronic uveitis, 78 132 Bartonella henselae, 240–243 laboratory tests for, 125t Bartonellosis Aspergillosis, 301 cat-­scratch disease, 240–243, 242f Aspergillus endophthalmitis, endogenous, 300–301 diagnosis of, 243 Autoantibodies, in systemic lupus erythematosus, 154 neuroretinitis associated with, 241, 242f Autocrine signaling, of cytokines, 21 ocular involvement in, 241, 242f Autofluorescence, fundus treatment of, 243 in acute posterior multifocal placoid pigment Basophils epitheliopathy, 163t, 171, 172f characteristics of, 2–3 in acute ret­i­nal pigment epitheliitis, 189 histamine in, 19 in acute zonal occult outer retinopathy, 163t, 191, Baylisascaris procyonis, 286 192f BCG vaccine, tuberculosis skin testing and, 238 Behçet disease Blurred vision anterior uveitis in, 214 in intermediate uveitis, 76 biologic agents for, 218 in posterior uveitis, 76 characteristics of, 211 Bone loss, corticosteroid-­induced, 103 corticosteroids for, 216–217 Borrelia burgdorferi, 227, 230 definition of, 211 Bradyzoites, 277 diagnosis of, 212, 212t, 215–216 Branch ret­i­nal artery occlusions, 160 differential diagnosis of, 216 B-­scan ultrasonography, for posterior scleritis, 124 fluorescein angiography findings in, 216, 217f Busacca nodules genital ulcers associated with, 213 in anterior uveitis, 78, 80f histologic findings in, 215, 216f in sarcoidosis, 196 HLA associations with, 215 ­human leukocyte antigen association with, 65t Calcific band keratopathy, in juvenile idiopathic immunomodulatory therapy for, 216–218 arthritis-­related anterior uveitis, 143f, 315 neurologic involvement in, 213–214 Cancer-­associated retinopathy, 192–193 nonocular systemic manifestations of, 213–214 Candida spp ocular manifestations of, 214–215, 214–215f C albicans, 296 optic nerve involvement in, 215 C glabrata, 296 optical coherence tomography findings in, 216, chorioretinitis caused by, 297, 298f 217f diagnosis of, 297 oral aphthae associated with, 213, 213f endogenous fungal endophthalmitis caused by, pathogenesis of, 215 295–299, 296f, 299f posterior segment manifestations of, 214–215, 215f histologic features of, 297, 298f prevalence of, 212 Candidemia, 296 prognosis for, 218 Candidiasis, ocular, 297–298 skin lesions associated with, 213 Candle wax drippings, 196, 197f systemic vasculitis associated with, 213 Can-­opener capsulotomy, 315 treatment of, 216–218 Carbonic anhydrase inhibitors, for uveitis, 113 Berlin nodules, 80 CARD15, 198 Beta chemokines, 23t Cataract(s) Bilateral diffuse uveal melanocytic proliferation, 309 in pars planitis, 151 Biopsy in uveitis, 313–317 chorioret­i­nal, in uveitis evaluations, 91 Cataract surgery scleral, 91 inflammation control ­after, 138 vitreous necrotizing scleritis ­after, 121 indications for, 91–92 in uveitic eyes, 313–314 technique for, 92 Cat-­scratch disease, 240–243, 242f Birdshot chorioretinopathy (BSCR) Cat-­scratch fever, 240 characteristics of, 162–164t CD8 course of, 168–169 in adaptive immune response, 30 definition of, 34, 165 cytotoxic T cell expression of, 36, 44 differential diagnosis of, 168 CD4, in adaptive immune response, 30 electroretinogram evaluations, 168 CD95 receptor, 46 fluorescein angiography findings in, 163t, 165–166, CD4+ T cells 166–167f in AIDS, 327 fundus autofluorescence findings in, 163t, 166, 167f development of, 43f HLA-­A29 and, 34, 64–65, 65t, 165 in immune pro­cessing, 30–31 indocyanine green angiography findings in, 163t, in sympathetic ophthalmia, 45 166, 167f, 179 in tubulointerstitial nephritis and uveitis syndrome, 135 manifestations of, 165–168, 166–168f CD8+ T cells optical coherence tomography findings in, 164t, 168, cytotoxicity mechanisms of, 47f 168f major histocompatibility class I molecules and, 30 posterior uveitis in, 165–169, 166–168f in sympathetic ophthalmia, 45 ret­i­nal pigment epithelium findings in, 166 Cefotaxime, for Lyme disease, 231t T helper cell-17 in, 34 Ceftriaxone, for Lyme disease, 231t treatment of, 169 Cefuroxime axetil, for Lyme disease, 231t Blau syndrome, 197 Cell adhesion molecules (CAMs) Blindness, uveitis as cause of, 68 description of, 10 Blood dendritic cells, 4 types of, 11 Blood-­derived macrophages, 3 Cell-­adhesion molecules, 38 Blood–­ocular barrier Cell-­mediated immunity, mast cells in, 3 cytomegalovirus passage across, 256 Central dysacusia, 205 description of, 56–57 Ce­re­bral vasculitis, 170 Cerebrospinal fluid, for syphilis testing, 225 Chronic fungal postoperative endophthalmitis, 292–293 Certolizumab, 110 Chronic inflammation, local antibody production in, 42 Chemokines Chronic peripheral rhegmatogenous ret­i­nal alpha, 23t detachment, 311 beta, 23t Chronic uveitis description of, 21 calcific band keratopathy associated with, 313 Chemotactic ­factors definition of, 75–76 definition of, 12 signs and symptoms of, 72t in neutrophil transmigration, 12 systemic corticosteroids for, 102–103 Chemotaxis, 2 Chronic Uveitis Evaluation of the Intravitreal Chickenpox, 247–248 Dexamethasone Implant study, 99t Chikungunya fever, 268f, 268–269 Churg-­Strauss syndrome, 125t ­Children Ciliary body, fenestrated capillaries in, 56 cytomegalovirus retinitis in, 255–256 Circinate balanitis, 133 intraocular lens in, 315 Circumcorneal conjunctival hyperemia, 232 uveitis in, 320 Class I-­dependent antigen-­presenting cells, 31f vision rehabilitation in, 325 Class II-­dependent antigen-­presenting cells, 30, 32f Chlorambucil, for uveitis, 110 Classic pathway activation, of complement, 17, 18f Choriocapillaris Clindamycin, for ocular toxoplasmosis, 281 immune responses of, 57–59 Coccidioides immitis, 301 immunologic microenvironment of, 57 Coccidioidomycosis, 301–302 immunoregulatory systems of, 59 Cogan syndrome, 125t Chorioret­i­nal biopsy, 91 Collagen vascular diseases Chorioret­i­nal lesions, 271, 272f polyarteritis nodosa, 156–158 Chorioret­i­nal scars, 135f systemic lupus erythematosus. See Systemic lupus Chorioret­i­nal spots, 264f erythematosus Chorioretinitis Collagenase, in inflammation, 25 acute syphilitic, 222, 222f Color photography, in uveitis evaluations, 89 Candida, 297, 298f Complement in Ebola virus survivor, 272f activation pathways for, 17, 18f posterior placoid, 222, 223f functions of, 17 relentless placoid, 173f, 173–174 in inflammation, 17–19 with vitritis, 72–73t in uveitis-­glaucoma-­hyphema syndrome, 10 without vitritis, 73t Complement receptors, 12 Choroid Congenital Epstein-­Barr virus infection, 257 immune responses of, 57–59 Congenital rubella syndrome, 258–259, 259f immunologic microenvironment of, 52t, 57–58 Congenital syphilis, 220, 226 immunoregulatory systems of, 59 Congenital toxoplasmosis, 276–277, 277f mast cells in, 58 Congenital Zika syndrome, 269 tuberculosis involvement of, 236–237f Conjunctiva Choroidal neovascularization (CNV) circumcorneal hyperemia of, 232 in multifocal choroiditis and panuveitis syndrome, effector cells in, 53 180f, 181 eosinophils in, 2 ocular histoplasmosis syndrome-­associated, 274 immunologic microenvironment of, 52t, 53 prevalence of, 327 immunoregulatory systems of, 53 in punctate inner choroiditis, 182 mast cells in, 3 serpiginous choroiditis and, 175 mucosa-­associated lymphoid tissue of, 53 treatment of, 325 sarcoidosis involvement of, 195, 195f in uveitis, 324–325 Conjunctivitis Choroidal scars, 274 in HIV patients, 334 Choroidal tubercles, 236, 237f in reactive arthritis syndrome, 133 Choroiditis rubella as cause of, 260 Cryptococcus neoformans, 333 solitary granulomatous, 334 macular, 274f Connective tissue mast cells, 2–3 multifocal. See Multifocal choroiditis; Multifocal Cornea choroiditis and panuveitis syndrome edema of, 54f Pneumocystis jirovecii, 332, 332f effector cells in, 54 punctate inner. See Punctate inner choroiditis immunologic microenvironment of, 52t, 54f, 54–55 serpiginous. See Serpiginous choroiditis immunoregulatory systems of, 54–55 serpiginous-­like, 177, 236, 237f Khodadoust line of endothelial rejection, 54, 54f Chronic anterior uveitis. See Anterior uveitis, chronic plasma-­derived proteins in, 54 Chronic Arthus reaction, 42 sarcoidosis involvement of, 196 Chronic cyclitis. See Pars planitis Corneal allograft rejection, 55 Corneal melting syndromes, 25 Cysticercus cellulosae, 285 Corticosteroids. See also specific drug Cytokines adverse effects of, 94, 103, 143, 150 in birdshot chorioretinopathy, 34 for autoimmune retinopathy, 193 definition of, 21 for Behçet disease, 216–217 growth ­factors, 21, 24t for cataracts, 314 in inflammation, 21, 22–24t for diffuse unilateral subacute neuroretinitis-­related intercellular signaling of, 21 inflammation, 288 interferons, 21, 24t for herpes simplex virus, 249 interleukins, 21, 22–23t for human­ T-lymphotropic virus type 1, 267 lipopolysaccharide effects on, 7 for iridocyclitis, 127 mast cell-­derived, 49 for Lyme disease-­related anterior segment ret­i­nal pigment epithelium synthesis of, 58 inflammation, 230 types of, 21, 22–24t for multifocal choroiditis and panuveitis syndrome, 181 Cytomegalovirus ocular hypertension caused by, 317, 319 anterior uveitis caused by, 248–249 for ocular toxoplasmosis, 281 Posner-­Schlossman syndrome caused by, 135 for pars planitis, 149–150 retinitis caused by posterior sub-­Tenon injection of, 95–96, 96f, 322 characteristics of, 254–257 for relentless placoid chorioretinitis, 173–174 in ­children, 255–256 for sarcoidosis, 199 in HIV/AIDS patients, 256, 328–329 for scleritis, 126 imaging of, 255f for sympathetic ophthalmia, 203 ret­i­nal detachments secondary to, 324, 329 for syphilitic uveitis-­related inflammation, 227 treatment of, 256–257 topical Cytoplasmic antinuclear antibody testing, 160 for iridocyclitis, 127 Cytotoxic T cells for uveitis, 94–95, 130 apoptosis inducement by, 46 for uveitis assassination by, 46 adverse effects of, 94, 103, 143, 150 autoimmunity and, 44 in ­children, 143 CD8 expression by, 36, 44 dose and duration of, 94, 102 cytotoxicity mechanisms of, 46, 47f intermediate, 149–150 description of, 44, 46 intravitreal, 97–98, 102 precursor, 46 local, 95 suicide induction by, 46 nonsteroidal anti-­inflammatory drugs and, 103 Cytotoxicity osteoporosis prevention strategies, 103 antibody-­dependent cellular, 47–48 periocular, 95–96, 96–97f CD8+ T cell, 47f phacoantigenic, 137 sustained-­release, 95 Daclizumab, for multiple sclerosis, 152 systemic, 102–103, 150 Dacryocystitis, 158 tapering of, 94 Dalen-­Fuchs nodules, 200, 202 topical, 94–95 Delayed hypersensitivity for visceral toxocariasis, 284 definition of, 42 Cortisol deficiency, corticosteroid-­induced, 94 granulomatous form of, 44 Cotton-­wool spots, 154, 155f, 256, 297, 327, 328f in scleritis, 116 COX-1. See Cyclooxygenase subtypes of, 42 COX-2. See Cyclooxygenase T helper cell-17 in, 44 Cryotherapy, for intermediate uveitis, 150 tuberculin form of, 28 Cryptococcosis, 299–300 Delayed hypersensitivity T cells Cryptococcus neoformans choroiditis, 333 description of, 36, 42–44 C-­type lectin receptors, 46 inflammatory diseases mediated by, 44t Curvilinear capsulorrhexis, 315 in sympathetic ophthalmia, 45–46 Cyclodestructive procedures, 320 T helper-­cell 2, 43 Cyclooxygenase (COX) in toxocara granuloma, 45 COX-1, 19 Dendritic cells COX-2, 19 characteristics of, 4–5 Cyclooxygenase (COX) pathway, in arachidonic acid major histocompatibility class II molecule expression oxidation, 19 by, 30 Cyclophosphamide, for uveitis, 109–110, 158, 160 pattern recognition receptors used by, 4 Cycloplegics, for uveitis, 113, 131 primed macrophages and, 14 Cyclosporine Dengue fever, 267f, 267–268 for Behçet disease, 217 Dexamethasone for uveitis, 108 intravitreal implant of, 97, 150 Cysticercosis, 285–286, 285–286f pellet formulation of, for uveitis, 97–98 Diffuse anterior scleritis, 116, 118 Endothelial graft rejection Diffuse unilateral subacute neuroretinitis (DUNS), epithelial edema with, 54f 287–288f, 287–289 Khodadoust line, 54, 54f Difluprednate, for uveitis, 94 stromal edema with, 54f Disease-­modifying antirheumatic drugs (DMARDs), Endotoxin. See also Lipopolysaccharide 103. See also Immunomodulatory therapy in innate immune response activation, 7 Doxycycline uveitis induced by, 8, 62 for bartonellosis, 243 Enhanced depth imaging optical coherence tomography for cat-­scratch disease, 243 in posterior scleritis, 124 for Lyme disease, 231t in uveitis, 85, 88f Drug-­induced uveitis, 139–140f, 139–141 Enteropathic arthritis, 125t Duodenal biopsy, for Whipple disease, 244 Enthesitis-­related arthritis (ERA), 142 Enzyme-­linked immunosorbent assay (ELISA) Eales disease, 237, 238f, 324 for leptospirosis, 232 Ebola virus, 270–271, 271f for syphilis, 225 Edema Eosinophils corneal, 54f characteristics of, 2 macular. See Macular edema granule products of, 2 optic disc, in bartonellosis, 241 Epiret­i­nal membranes, 323 Effector blockade, 57 Episcleritis Effector lymphocytes, 36 nonsteroidal anti-­inflammatory drugs for, 113 Eicosanoids scleritis versus, 117 in inflammation, 19–20, 20f Epithelioid cells, macrophage activation into, 4 posterior diffusion of, 19–20 Epitope(s) schematic diagram of, 20f in adaptive immune response, 27, 30 Electroretinography antigenic, 27, 41 birdshot chorioretinopathy evaluations, 168 definition of, 27 retinitis pigmentosa evaluations, 310 idiotopes versus, 41 uveitis evaluations, 90 Epstein-­Barr virus, 257–259 Elschnig spots, 157 Equine recurrent uveitis, 62 Endocrine signaling, of cytokines, 21 Erythema chronicum migrans, 227–228, 228f Endophthalmitis E-­Selectin, 11–12 acute postoperative, 291–292 Etanercept, for sarcoidosis, 199 bacterial toxin induction in, 9 Ethylenediaminetetraacetic acid, 313 Candida, 295–299, 296f, 299f Exciting eye, 199–200 chronic postoperative Exotoxins bacterial ­causes of, 292 definition of, 9 clinical findings of, 292, 292f innate immune response triggered by, 9 diagnosis of, 293 Experimental autoimmune uveoretinitis (EAU), 61–62 fungal ­causes of, 292–293 Nd:YAG capsulotomy triggering of, 292 Fab fragment, of B cells, 41 overview of, 291–292 Famciclovir, for herpes simplex virus, 249 Propionibacterium acnes as cause of, 292, 292f Fas ligand treatment of, 293 apoptosis induced by, 57 definition of, 291 in effector blockade, 57 endogenous Fc receptors bacterial, 294–295 antibody, 12 fungal, 295–302, 296f, 298f, 300f description of, 3 fungal mast-­cell, 19 Aspergillus as cause of, 300–301 types of, 47 Candida, 295–299, 296f, 299f Fibrin coccidioidomycosis as cause of, 301–302 dissolution of, 18 cryptococcosis as cause of, 299–300 in inflammation, 18–19 endogenous, 295–302, 296f, 298f, 300f in uveitis-­glaucoma-­hyphema syndrome, 10 manifestations of, 295 Fibrinogen, 18 “string of pearls” in, 296f Fingolimod, for multiple sclerosis, 152 hypopyon of, 6 Floaters, in intermediate uveitis, 76 infectious, acute anterior uveitis associated with, Fluconazole, for coccidioidomycosis, 302 137 Fluocinolone acetonide implant innate immune response in, 5 for birdshot chorioretinopathy, 169 Endoplasmic reticulum aminopeptidase (ERAP), for multifocal choroiditis and panuveitis syndrome, 65 181 Endothelial cells, activation of, 10, 12 for sympathetic ophthalmia, 203 for uveitis Fungal uveitis description of, 97–98 ocular histoplasmosis syndrome, 272–275, 273–274f macular edema caused by, 322 polymerase chain reaction for, 90 Fluorescein angiography (FA) in acute posterior multifocal placoid pigment Ganciclovir epitheliopathy, 163t, 170, 171f for cytomegalovirus, 256 in acute ret­i­nal pigment epitheliitis, 189, 189f for herpes simplex virus, 252 in acute zonal occult outer retinopathy, 163t, 191 GATA3 transcription ­factor, 32 in Behçet disease, 216, 217f Gene therapy, ret­i­nal, 58 in birdshot chorioretinopathy, 163t, 165–166, Genotyping, 63 166–167f ­Giant cell arteritis, 125t in Dengue fever, 267 ­Giant cells, macrophage activation into, 4 in granulomatous necrotizing scleritis, 120f Glaucoma in intermediate uveitis, 148 angle-­closure, secondary, 318 in multifocal choroiditis and panuveitis syndrome, corticosteroid-­induced, 319 163t, 179, 179f open-­angle, secondary, 318–319 in multiple evanescent white dot syndrome, 163t, in pars planitis, 151 185, 186f phacolytic, 13, 137 in ocular ischemic syndrome, 310 uveitic, 317 in pars planitis, 148 optical coherence tomography of, 85 in primary vitreoret­i­nal lymphoma, 305 Glaucoma filtering surgery, 319 in punctate inner choroiditis, 163t, 182, 183f Glaucomatocyclitic crisis, acute anterior uveitis in, in serpiginous choroiditis, 163t, 175f 135–136 in subret­i­nal fibrosis and uveitis syndrome, 163t, 184 Goldmann-­Witmer (GW) coefficient in sympathetic ophthalmia, 200, 201f calculation of, 41–42 in tubercular uveitis, 236, 237f description of, 251–252 in uveitis, 88, 89f for Fuchs heterochromic uveitis, 41–42 in Vogt-­Koyanagi-­Harada syndrome, 208–209, 209f polymerase chain reaction and, 91 in West Nile virus, 263, 264f for toxoplasmosis, 91 Fluo­rescent treponemal antibody absorption assay Golimumab, 110 (FTS-­ABS), 224–225 Gomori methenamine silver stain, 298f Foreign bodies, intraocular, 311 Goniotomy, 320 Foscarnet Granular cytomegalovirus retinitis, 255f for cytomegalovirus, 256 Granuloma for herpes simplex virus, 252 iris, 300f, 302 “Frosted-­branch” angiitis, 254, 255f, 304 toxocara, 45 Fuchs heterochromic uveitis Granulomatosis with polyangiitis (GPA) Goldmann-­Witmer coefficient for, 41–42 definition of, 158 keratic precipitates in, 248 diagnosis of, 159–160 medical management of, 93 laboratory investigations for, 125t Fuchs uveitis syndrome, 145–146, 146f manifestations of, 158–159, 159f Fundus autofluorescence necrotizing scleritis in, 120f in acute posterior multifocal placoid pigment ocular involvement in, 159 epitheliopathy, 163t, 171, 172f treatment of, 160 in acute ret­i­nal pigment epitheliitis, 189 vision loss in, 159 in acute zonal occult outer retinopathy, 163t, 191, 192f Granulomatous necrotizing scleritis, 120f, 120–121 in birdshot chorioretinopathy, 163t, 166, 167f Growth ­factors in multifocal choroiditis and panuveitis syndrome, definition of, 21 163t, 179, 180f 186–187f types of, 24t in multiple evanescent white dot syndrome, 163t, 186f Haplotype, 63 in punctate inner choroiditis, 163t Haze (vitreous), in intermediate uveitis, 81f, in serpiginous choroiditis, 163t, 176f 81–82, 82t in subret­i­nal fibrosis and uveitis syndrome, 163t Heerfordt syndrome, 195 in uveitis evaluations, 89 Helper T cells Fungal endophthalmitis, endogenous accessory molecules expressed by, 31 Aspergillus as cause of, 300–301 in adaptive immune response, 30, 32f Candida, 295–299, 296, 299f antigen receptor on, 30 coccidioidomycosis as cause of, 301–302 classification of, 32 cryptococcosis as cause of, 299–300 differentiation of, 32–35 endogenous, 295–302, 296f, 298f, 300f priming of, 31 manifestations of, 295 subsets of, 32 “string of pearls” in, 296f Th1, 32–33 Th2, 32 ­Human T-lymphotropic virus type 1 (HTLV-1) Th17, 33–34 description of, 265–267, 266f Hemorrhage, intraret­i­nal, 157f keratopathy associated with, 266 Herpes simplex virus (HSV) Hutchinson sign, 248 anterior uveitis associated with, 247–250 Hutchinson triad, 220 infectious scleritis caused by, 124 Hyalocytes, 56 keratitis associated with, 334 Hydrogen peroxide, 16 ocular hypertension associated with, 248–249 Hydroxyl radical, 16 Herpes zoster, 248, 334 Hyperemia, in scleritis, 117, 117f Herpes zoster ophthalmicus, 248 Hypersensitivity Herpetic choroiditis, 177 delayed Herpetic encephalitis, 251 definition of, 42 Herpetic retinitis granulomatous form of, 44 necrotizing, 330, 330f subtypes of, 42 nonnecrotizing, 250–254 T cells Heterochromia, in Fuchs uveitis syndrome, 145, 146f description of, 36, 42–44 Histamine inflammatory diseases mediated by, 44t in basophils, 19 in sympathetic ophthalmia, 45–46 in connective tissue mast cells, 3 T helper-­cell 2, 43 in mast cells, 19 in toxocara granuloma, 45 in mucosal mast cells, 3 T helper cell-17 in, 44 Histo spots, 273, 273f tuberculin form of, 28 Histoplasma capsulatum, 272 immediate, 48–49 HIV. See also AIDS Hypopyon cytomegalovirus retinitis in, 256 in acute anterior uveitis, 130, 131f herpes zoster and, 334 in Aspergillus endophthalmitis, 300 ocular toxoplasmosis in, 281–282, 282f in Behçet disease, 214, 214f ophthalmic manifestations of, 327–333, 328f, 330f, 332f endophthalmitis, 6 retinopathy associated with, 327, 328f lens-­associated uveitis, 6 syphilis and, 219–220, 331–332 in leukemia, 308 HLA-­A29 Hypotony, 321 birdshot chorioretinopathy and, 34, 64–65, 65t, 165 prevalence of, 64 Idiopathic stellate maculopathy, 241 HLA-­B12, 215 Idiotopes, 41 HLA-­B27 Immediate hypersensitivity, 48–49 characteristics of, 131 Immune checkpoint inhibitors, Vogt-­Koyanagi-­Harada diseases associated with syndrome caused by, 140f ankylosing spondylitis, 130f, 131–132 Immune privilege clinical pre­sen­ta­tion of, 130, 130f in cornea, 54 inflammatory bowel syndrome, 133 definition of, 51 pattern of, 130 Immune pro­cessing psoriatic arthritis, 133, 134f antigen in, 30, 33f reactive arthritis syndrome, 132–133, 133f CD4+ T cells in, 30–31 uveitis and, 64, 65t Immune recovery uveitis (IRU), 329 HLA-­B51, 215 Immune response(s) HLA-­D, 63 adaptive. See Adaptive immune response/adaptive HLA-­DM, 63 immunity HLA-­DR4 of anterior chamber, 55–57 Lyme disease and, 228 of anterior uvea, 55–57 Vogt-­Koyanagi-­Harada syndrome and, 204 of choriocapillaris, 57–59 Homing, 38 of choroid, 57–59 ­Human granulocytic anaplasmosis, 230 of conjunctiva, 53 ­Human granulocytic ehrlichiosis, 230 of cornea, 54f, 54–55 ­Human herpesvirus 8, 333 definition of, 1, 6 ­Human immunodeficiency virus. See HIV inflammation versus, 6 ­Human leukocyte antigens. See also specific HLA innate. See Innate immune response/innate immune allelic variation, 63–64 system disease associations with, 64–66, 65t mediator systems that amplify function of, 63 complement, 17–19 genotyping of, 63 eicosanoids, 19–20, 20f haplotype of fibrin, 18–19 definition of, 63 histamine, 18 disease associations with, 64–66, 65t overview of, 17 plasma-derived­ enzyme systems, 17–19 biologic agents, 104, 110–113 plasminogen, 18 chlorambucil, 110 platelet-­activating ­factors, 20–21 complications of, 105 neutrophils in, 2 cyclophosphamide, 109–110 primary, 37–38 cyclosporine, 108 regional immunity and, 51, 52t description of, 103 of ret­ina, 57–59 evaluations before initiating, 104–105 of ret­i­nal pigment epithelium, 57–59 indications for, 104 secondary, 37–38 infliximab, 111–112 of vitreous, 55–57 interferon alfa-2a/2b, 112 Immune response arc methotrexate, 107 mucosa-­associated lymphoid tissue, 53 monitoring during, 105 phases of, 28–37, 29f, 31–33f mycophenolate mofetil, 107 afferent, 29f, 29–30 nonbiologic agents, 104, 106–110 effector, 36–37 onset of action, 105 pro­cessing, 30–35, 33f pregnancy contraindications for, 105 regional immunity and, 51, 52t in psoriatic arthritis, 133 Immune system rituximab, 112 components of, 2–5 tacrolimus, 108 leukocytes of. See Leukocytes T-­cell inhibitors, 108–1109 Immunoglobulin(s). See also Antibodies tumor necrosis ­factor inhibitors, 105, 110–112, classes of, 39 130–131 description of, 11 Immunoregulatory systems functional properties of, 39, 41 of anterior chamber, 56–57 isotypes of, 39, 40t of anterior uvea, 56–57 plasma cell production of, 37 of choriocapillaris, 59 structural features of, 39, 39f of choroid, 59 Immunoglobulin A of conjunctiva, 53 in conjunctiva, 53 of cornea, 54–55 molecular size of, 39 of ret­ina, 59 Immunoglobulin A1, 41 of ret­i­nal pigment epithelium, 59 Immunoglobulin A2, 41 of vitreous, 56–57 Immunoglobulin E Indocyanine green angiography (IGA) inflammatory reactions mediated by in acute posterior multifocal placoid pigment Fc receptors for, 3 epitheliopathy, 163t, 170–171, 171f mast cells in, 3 in acute zonal occult outer retinopathy, 163t mast-­cell degranulation mediated by, 48–49 in birdshot chorioretinopathy, 163t, 166, 167f, 179 Immunoglobulin G4, 41 in granulomatous necrotizing scleritis, 120f Immunoglobulin M in multifocal choroiditis and panuveitis syndrome, description of, 35 163t molecular size of, 39 in multiple evanescent white dot syndrome, 163t, 188f primary immune response production of, 37 in punctate inner choroiditis, 163t, 182, 183f Immunologic microenvironment in serpiginous choroiditis, 163t, 175 of anterior chamber, 55–56 in subret­i­nal fibrosis and uveitis syndrome, 163t of anterior uvea, 55–56 in sympathetic ophthalmia, 201, 202f of choriocapillaris, 57–58 in tubercular uveitis, 236, 237f of choroid, 57–58 in uveitis, 89 of conjunctiva, 52t, 53 in Vogt-­Koyanagi-­Harada syndrome, 209 of cornea, 54f, 54–55 Inducible nitric oxide synthetase (iNOS), 16 of ret­ina, 57–58 Inducible T regulatory cells, 34 of ret­i­nal pigment epithelium, 52t, 57–58 Infectious endophthalmitis, acute anterior uveitis of vitreous, 52t, 55–56, 56 associated with, 137 Immunomodulatory therapy (IMT) Infectious scleritis for Behçet disease, 216–218 actinomycetes as cause of, 119f for birdshot chorioretinopathy, 169 clinical pre­sen­ta­tion of, 124 for sarcoidosis, 199 description of, 116 for scleritis, 126 microbiological examination for, 125 for uveitis pathophysiology of, 123–124 abatacept, 112 treatment of, 127 adalimumab, 110–111 vision loss risks, 128 alkylating agents, 105, 109–110 Infectious uveitis antimetabolites, 106–108 acute ret­i­nal necrosis, 250, 251f azathioprine, 106–107 adenovirus as cause of, 272 bartonellosis as cause of. See Bartonellosis Inflammatory dendritic cells, 4 Chikungunya fever as cause of, 268–269 Inflammatory response cytomegalovirus as cause of, 248–249, 254–257, clinical manifestations of, 6 255f definition of, 6 Dengue fever as cause of, 267f, 267–268 lipopolysaccharide-­induced, 8–9 Ebola virus as cause of, 270–271, 271f Infliximab Epstein-­Barr virus as cause of, 257–258 anti-­idiotypic antibodies’ effect on, 41 herpes simplex virus as cause of, 247–250, 248–249f for Behçet disease, 216 ­human T-lymphotropic virus type 1 as cause of, sarcoidosis-­associated uveitis treated with, 199 265–267, 266f for uveitis, 111–112 immunologic uveitis versus, 311 Innate immune response/innate immune system leptospirosis as cause of, 232–233 adaptive immune response versus, 27 Lyme disease as cause of. See Lyme disease conjunctiva and, 53 lymphocytic choriomeningitis virus as cause definition of, 1 of, 261 macrophage activation by, 14 measles as cause of, 261–263 overview of, 5 mumps as cause of, 261 subclinical presence of, 6 nocardiosis as cause of, 233 summary of, 7t periocular injection contraindications in, 96 triggers of progressive outer ret­i­nal necrosis, 250–254, 253f bacterial-­derived molecules, 7t, 7–9 rhegmatogenous ret­i­nal detachment associated exotoxins, 9 with, 324 lipopolysaccharide, 7–8 Rift Valley fever as cause of, 265, 265f overview of, 6–7, 7t rubella as cause of, 258–261 Integrins, 11 subacute sclerosing panencephalitis as cause of, Intercellular adhesion molecule-1 (ICAM-1), 11 262–263 Intercellular signaling, of cytokines, 21 syphilis as cause of. See Syphilis Interferon(s) tuberculosis as cause of, 234–240, 235–238f. See also definition of, 21 Tuberculosis types of, 24t West Nile virus as cause of, 263–264 Interferon-­, 24t Whipple disease as cause of, 243–245, 244f Interferon alfa-2a/2b Zika fever as cause of, 269–270, 270f for Behçet disease, 218 Inferior transseptal injection, of periocular for uveitis, 112 corticosteroids, 96, 97f Interferon-­gamma Inflammation characteristics of, 24t cytokines in, 21, 22–24t macrophage activation by, 42 eicosanoids in, 19–20, 20f T helper cell-1 production of, 35 immune response versus, 6 Interferon-­gamma release assays, tuberculosis testing leukotrienes in, 20 using, 29 pathologic findings associated with, 6 Interleukin(s) Inflammatory bowel disease, acute anterior uveitis definition of, 21 in, 133 types of, 22–23t Inflammatory cells, in anterior uveitis, 77, 79f Interleukin-1, 22t Inflammatory chorioretinopathies Interleukin-2 acute posterior multifocal placoid pigment characteristics of, 22t epitheliopathy. See Acute posterior multifocal T cell synthesis and release of, 31 placoid pigment epitheliopathy Interleukin-4 acute ret­i­nal pigment epitheliitis, 162–164f, 189f, in B-­cell activation, 35, 43 189–190 characteristics of, 22t acute zonal occult outer retinopathy. See Acute zonal Interleukin-5 occult outer retinopathy characteristics of, 22t birdshot chorioretinopathy. See Birdshot eosinophils and, 2 chorioretinopathy Interleukin-6 definition of, 161 characteristics of, 22t multifocal choroiditis and panuveitis syndrome. See in inflammatory uveitis, 306 Multifocal choroiditis and panuveitis syndrome Interleukin-8, 23t multiple evanescent white dot syndrome. See Multiple Interleukin-10 evanescent white dot syndrome in intraocular lymphoma, 306 punctate inner choroiditis. See Punctate inner in T regulatory cell effector function, 35 choroiditis Interleukin-12 serpiginous choroiditis. See Serpiginous choroiditis antigen-­presenting cell production of, 35 subret­i­nal fibrosis and uveitis syndrome. See Subret­i­ characteristics of, 23t nal fibrosis and uveitis syndrome Interleukin-17A, 23t Intermediate uveitis Ixodes pacificus, 227 characteristics of, 70 Ixodes scapularis, 227 chronic, 82 conditions associated with, 147 Jarisch-­Herxheimer reaction, 226, 230 evaluation of, 72t Juvenile idiopathic arthritis (JIA) floaters in, 76 calcific band keratopathy in, 143f, 313 ­human leukocyte antigen association with, 65t chronic anterior uveitis in in ­human T-lymphotropic virus type 1, 266 description of, 76, 141–145 immune recovery and, 257 methotrexate for, 107 in Lyme disease, 229 classification of, 141–142 in multiple sclerosis, 151–152 differential diagnosis of, 142 pars planitis. See Pars planitis ­human leukocyte antigen association with, 65t peripheral vascular sheathing in, 75 laboratory tests for, 125t signs of, 81–82, 81–82f ocular involvement in, 141–142, 143f snowball opacities in, 82, 147, 148f oligoarticular, 142 snowbanks in, 82, 147 polyarticular, 141–142 symptoms of, 76, 77t prognosis for, 142 vitreous cells in, 81–82, 82t treatment of, 142–145 vitreous haze in, 81f, 81–82 Juvenile xanthogranuloma, 309 International Study Group for Behçet disease, 212, 212t Kaposi sarcoma, 333, 333f Interphotoreceptor retinoid-­binding protein-­specific Keratic precipitates T-­cell receptor transgenic mice, 62 in anterior uveitis, 77–78, 79f Interstitial keratitis, 220, 221f in Fuchs heterochromic uveitis, 248 Intraepithelial lymphocytes, 5 in Fuchs uveitis syndrome, 146f Intraocular foreign bodies, 311 mutton-­fat, 78, 79f, 196 Intraocular lens (IOLs) in sarcoidosis, 196, 196f anterior chamber in Vogt-­Koyanagi-­Harada syndrome, 205, 207 anterior uveitis caused by, 138, 138f Keratitis fixed-­haptic, 138f herpes simplex, 334 biocompatibility of, 138 in Lyme disease, 229 in ­children, 315 punctate epithelial, 334f in juvenile idiopathic arthritis-­associated uveitis Keratoderma blennorrhagicum, 133, 134f cataracts, 315 Keratouveitis, 70, 220, 247 phacoemulsification with, 315 Khodadoust line of endothelial rejection, 54, 54f retained fragments of, 90 Killer cells, 48 uveitis associated with, 137–139, 138f Killer immunoglobulin-­like receptors, 46 uveitis-­glaucoma-­hyphema syndrome associated Koeppe nodules with, 10, 137 in anterior uveitis, 78 Intraocular pressure. See also Ocular hypertension in sarcoidosis, 196, 196f in anterior uveitis, 80–81, 317 Krill disease. See Acute ret­i­nal pigment epitheliitis increased Kupffer cells, 3 difluprednate as cause of, 94 Kyrielesis arteriolitis, 278 periocular corticosteroids as cause of, 96 Intraret­i­nal hemorrhage, 157f Lacrimal gland, sarcoidosis involvement of, 195 Intravenous immunoglobulin, for uveitis, 113 Langerhans cells Intravitreal corticosteroids, for uveitis, 97–98, 102 characteristics of, 4–5 Intravitreal cysts, 286f functions of, 4–5 Iridocyclitis ­Laser photocoagulation, for intermediate uveitis, 150 corticosteroids for, 127 Leber congenital amaurosis, 58 definition of, 70 Leber idiopathic stellate maculopathy, 241 Iridolenticular diaphragm, 123 Lens-­associated uveitis Iris bombé, 81, 130, 196 hypopyon in, 6 Iris chafing, 137 types of, 136f, 136–137 Iris granulomas, 300f, 302 Leptospira interrogans, 232 Iris heterochromia, in Ebola virus, 271f Leptospirosis, 232–233 Iris nodules Leukemia, neoplastic masquerade syndromes secondary in anterior uveitis, 77–78, 80, 80f to, 308 in ocular tuberculosis, 235f Leukocytes Iris stromal atrophy, 249f basophils, 2–3 Iritis, 70 classification of, 2 Isoniazid, for tuberculosis, 239–240 cytokine effects on, 25 Ivermectin, for onchocerciasis, 290 dendritic cells, 4–5 eosinophils, 2 age of onset, 303 Fc receptor expression by, 47 clinical features and findings of, 304–305, 304–305f Langerhans cells, 4–5 diagnostic testing for, 305–306 lymphocytes, 5 fluorescein angiography findings in, 305 macrophages, 3–4 histology of, 306, 307f mast cells, 2–3 incidence of, 303 monocytes, 3–4 Lysosomes neutrophils, 2 in eosinophils, 2 oxygen metabolism by, 16 in neutrophils, 2 platelet-­activating ­factor activation of, 21 Leukocytoclastic vasculitis, 125t Macrolides, for Lyme disease, 231t Leukotrienes, in inflammation, 20 Macrophage(s) Linkage disequilibrium, 64 activation of, 13–16, 15f, 21 Lipofuscin, 89 as antigen-­presenting cells, 14 Lipopolysaccharide (LPS) characteristics of, 3–4 animal models of, 8 epithelioid cell activation of, 4 components of, 7 functions of, 3 gut release of, 8 ­giant cell activation of, 4 inflammatory response induced by injection of, 8–9 interferon-­gamma activation of, 42 in innate immune response activation, 7–8 major histocompatibility class II molecule expression in neutrophil activation, 12 by, 30 ocular effects induced by, 8 mediators synthesized by, 15 Lipoxin, 20 primed, 14, 15f 5-­Lipoxygenase recruitment of, 13–16 in arachidonic acid oxidation, 19 reparative, 16 derivatives of, 20 resting, 13, 15f Loa loa, 290 scavenging, 13 Löfgren syndrome, 195 stimulated, 14–16, 15f L-­Selectin, 11–12 tuberculosis response by, 28 Lupus choroidopathy, 154, 155f Macrophage chemotactic protein-1, characteristics Lupus retinopathy, 154, 155f of, 23t Lyme disease Macrophage-­activating ­factor, 42 antibiotics for, 230, 231t Macular choroiditis, 274f clinical features of, 227–229, 228f Macular edema corticosteroids for, 230 in intermediate uveitis, 70 definition of, 227 nonsteroidal anti-­inflammatory drugs for, 113 diagnosis of, 230 in pars planitis, 147 epidemiology of, 227 in tubulointerstitial nephritis and uveitis syndrome, erythema chronicum migrans associated with, 134 227–228, 228f uveitic HLA-­DR4 and, 228 description of, 19 keratitis in, 229 fluorescein angiography of, 88, 89f neuro-ophthalmic­ manifestations of, 229 optical coherence tomography of, 85, 88f ocular involvement in, 229, 229f in phacoemulsification, 316 stage 1, 227–228, 228f postoperative, 316 stage 2, 228 smoking as risk ­factor for, 321 stage 3, 228–229 treatment of, 322 treatment of, 230, 231t Macular hole, 323 vitritis in, 229, 229f Major histocompatibility class I molecules Lymphatics, 30 CD8+ T cells and, 30 Lymphocytes definition of, 63 activation of, 30 Major histocompatibility class II molecules B. See B cells cellular expression of, 30 characteristics of, 5 definition of, 63 effector, 36 expression of, 4 homing of, 38 Langerhans cell expression of, 5 sensitization of, 30 ret­i­nal pigment epithelium expression of, 57 surface markers of, 5 Mannose-­binding lectin pathway, 17, 18f T. See T cells Masquerade syndromes, 84 Lymphocytic choriomeningitis virus (LCMV), 261 chronic peripheral rhegmatogenous ret­i­nal Lymphoma detachment, 311 primary central ner­vous system, 303, 304f intraocular foreign bodies, 311 primary vitreoret­i­nal juvenile xanthogranuloma, 309 leukemia as cause of, 308 Mucosa-­associated lymphoid tissue (MALT), 53 metastatic tumors as, 309 Mucosal mast cells, 2–3 neoplastic, 303–309 Multicenter Uveitis Ste­roid Treatment Trial, 98, 99t nonneoplastic, 310–311 Multidrug-­resistant tuberculosis (MDRTB), 239 ocular ischemic syndrome, 310 Multifocal chorioretinitis pigment dispersion syndrome, 311 in West Nile virus, 263 primary vitreoret­i­nal lymphoma. See Primary in Whipple disease, 244, 244f vitreoret­i­nal lymphoma Multifocal choroiditis retinitis pigmentosa, 310 in AIDS patients, 332 retinoblastoma, 308–309 peripheral, 229 systemic lymphoma as cause of, 307 in systemic lupus erythematosus, 155f uveal lymphoid proliferations, 308 tubercular, 236f uveal melanoma, 308 Multifocal choroiditis and panuveitis syndrome (MCP) Mast cells characteristics of, 162–164t, 177–178 acute immunoglobulin E-­mediated degranulation of, definition of, 178 48–49 diagnosis of, 181 characteristics of, 2–3 fluorescein angiography findings in, 163t, 179, 179f in choroid, 58 fundus autofluorescence findings in, 163t, 179, 180f in conjunctival substantia propria, 3, 53 lesions in, 178, 178f connective tissue, 2–3 manifestations of, 178–180f, 178–181 cytokines derived from, 49 optical coherence tomography findings in, 164t, 179, histamine in, 19 180f mucosal, 2–3 prognosis for, 181 Measles, 261–263 treatment of, 181 Mediators Multifocal serpiginous choroiditis, 177 reactive nitrogen products, 16 Multiple evanescent white dot syndrome (MEWDS) reactive oxygen intermediates, 16 acute macular neuroretinopathy and, 189 Melanoma characteristics of, 162–164t metastatic, 309 definition of, 185 uveal, 308 description of, 161 Melanoma-­associated retinopathy, 192–193 diseases associated with, 189 Memory cells, 37 early receptor potential amplitudes in, 187 6-­Mercaptopurine, 106 fluorescein angiography findings in, 163t, 185, Metamorphopsia, 273 186–187f Metastatic melanoma, 309 fundus autofluorescence findings in, 163t, 186–187f, Metastatic tumors, 309 187 Methotrexate, for uveitis indocyanine green angiography findings in, 163t, adverse effects of, 107 188f dosing of, 107 manifestations of, 185–188, 185–188f treatment studies involving, 100t medical management of, 93 Methylprednisolone prognosis for, 188 for scleritis, 126–127 Multiple sclerosis for uveitis, 95, 96f, 102–103 ­human leukocyte antigen association with, 65t Micafungin, for Candida fungal postoperative intermediate uveitis associated with, 151–152 endophthalmitis, 299 Mumps, 261 Microglia, 3 Murine models 2-­Microglobulin, 31f autoimmune regulator–­deficient mice, 62 Microhemagglutination assay for T. pallidum antibodies, interphotoreceptor retinoid-­binding protein-­specific 224 T-­cell receptor transgenic mice, 62 Microscopic polyangiitis (MPA), 125t, 158 Mutton-­fat keratic precipitates, 78, 79f, 196 Migratory dendritic cells, 4 Mycobacteria tuberculosis. See also Tuberculosis Miliary disease, 234 description of, 234 Mitomycin C, 319 interferon-­gamma release assays for, 29 Molluscum contagiosum, 334 nucleic acid amplification techniques for, 239 Monoclonal antibodies serpiginous-­like choroiditis caused by, 177 description of, 41 Mycophenolate mofetil, for uveitis, 107 recombinant, 41 Mydriatics, for uveitis, 113 Monocytes Myeloperoxidase, 158, 160 as antigen-­presenting cells, 3 characteristics of, 3–4 National Institutes of Health vitreous haze grading lipopolysaccharide effects on, 7 system, 82 Mononeuritis multiplex, 158 Natu­ral killer (NK) cells, 5, 46 Moxifloxacin, 139 Naturally occurring T regulatory cells, 34 Nd:YAG capsulotomy Ocular adnexal Kaposi sarcoma, 333, 333f chronic postoperative endophthalmitis triggered Ocular histoplasmosis syndrome (OHS), 272–275, by, 292 273–274f uveitis and, 316 Ocular hypertension. See also Intraocular pressure Necrotizing herpetic retinitis, 330, 330f corticosteroid-­induced, 317, 319 Necrotizing retinopathies herpes simplex virus and, 248–249 acute ret­i­nal necrosis, 250, 251–252f uveitic, 317 cytomegalovirus-­related anterior uveitis, 248–249 Ocular immune privilege definition of, 250 antigens, 56 progressive outer ret­i­nal necrosis, 250–254 definition of, 51 Necrotizing scleritis regulators involved in, 57 anterior, 118f Ocular ischemic syndrome, 310 clinical pre­sen­ta­tion of, 118f, 119–122 Ocular nocardiosis, 233 delayed hypersensitivity response in, 116 Oligoarthritis, 143f description of, 115 Onchocerca volvulus, 289 diagnosis of, 124 Onchocerciasis, 289f, 289–290 granulomatous, 120f, 120–121 Open-­angle glaucoma, secondary, 318–319 with inflammation, 120–121, 120–121f Opportunistic infections laboratory tests for, 124 in AIDS patients, 328 mortality rates for, 119 during cyclophosphamide treatment, 109 pathophysiology of, 116 Optic disc edema, in bartonellosis, 241 postsurgical, 121, 121f Optic nerve vaso-­occlusive, 120, 121f atrophy of, 269, 270f vision loss risks, 128 Behçet disease involvement of, 215 without inflammation, 121–122, 122f Optic neuritis, 223 Neovascularization Optical coherence tomographic angiography (OCTA), choroidal. See Choroidal neovascularization in uveitis, 87 ret­i­nal, in uveitis, 324–325 Optical coherence tomography (OCT) Neuropeptides, 24t in acute posterior multifocal placoid pigment Neuroretinitis epitheliopathy, 164t, 171, 172f bartonellosis as cause of, 241, 242f in acute ret­i­nal pigment epitheliitis, 189 differential diagnosis of, 242t in acute zonal occult outer retinopathy, 164t, 191, 191f diffuse unilateral subacute, 287–288f, 287–289 anterior segment syphilis versus, 223 indications for, 85 Neurosyphilis, 226 in uveitis evaluations, 85 Neutrophil(s) in Behçet disease, 216, 217f activation of, 10, 11f, 12 in birdshot chorioretinopathy, 164t, 168, 168f adhesion of, 11f, 12 in Dengue fever, 267, 267f characteristics of, 2 enhanced depth imaging granule products of, 25 in posterior scleritis, 124 in innate immune response, 10–12 in uveitis, 85, 88f lipopolysaccharide effects on, 7 in macular edema, uveitic, 85, 88f, 321 recruitment and activation of, 10–12, 11f in multifocal choroiditis and panuveitis syndrome, resting, 10 164t, 179, 180f transmigration of, 11f, 12 in multiple evanescent white dot syndrome, 164t Neutrophil rolling, 10, 11f in posterior scleritis, 123f Nitric oxide, 16 in punctate inner choroiditis, 164t, 182, 183f Nitric oxide synthetase (NOS), 16 in serpiginous choroiditis, 164t, 175, 176f Nivolumab, Vogt-­Koyanagi-­Harada syndrome caused spectral-­domain, for posterior scleritis, 124–125 by, 140f in subret­i­nal fibrosis and uveitis syndrome, 164t, 184 Nocardia asteroides, 233, 311 sympathetic ophthalmia findings in, 201–202, 202f Nocardiosis, 233 uveitis evaluations, 85, 88f Nodular anterior scleritis, 116, 118–119, 119f in Vogt-­Koyanagi-­Harada syndrome, 209, 210f Nonnecrotizing herpetic retinitis, 250–254 Orbital cellulitis, 158 Nonnecrotizing herpetic retinopathy, 250 Orbital pseudotumor, 158 Nonsteroidal anti-­inflammatory drugs (NSAIDs) Osteoporosis, corticosteroid-­induced, 103 ankylosing spondylitis treated with, 132 Oxygen, leukocyte metabolism of, 16 corticosteroids and, concomitant use of, 103 macular edema treated with, 322 Panuveitis scleritis treated with, 126 characteristics of, 75 uveitis treated with, 113, 315 definition of, 194 Nonulcerative stromal interstitial keratitis, 220, 221f in Ebola virus, 271f Nozik technique, for uveitis, 95–96, 97f evaluation of, 71t rhegmatogenous ret­i­nal detachment associated with, Phacoantigenic uveitis, 136–137 324 Phacolytic glaucoma, 13 in sarcoidosis, 194–199 Phacolytic uveitis, 136f, 136–137 in sympathetic ophthalmia, 199–203 Phagocytosis in Vogt-­Koyanagi-­Harada syndrome. See Vogt-­ antigen-­presenting cells in, 30, 32f Koyanagi-­Harada (VKH) syndrome description of, 12 Paracentesis, anterior chamber mechanisms of, 16 in bacterial endogenous endophthalmitis, 295 reactive oxygen intermediates in, 16 technique for, 92 Phagosomes, 12 in uveitis, 90–92 Phospholipase A2 Paracrine signaling, of cytokines, 21 arachidonic acid from, 19 Paratope, 35 functions of, 21 Parinaud oculoglandular syndrome, 241 Phospholipids, arachidonic acid from, 19 Pars plana vitrectomy Pigment dispersion syndrome, 311 chronic postoperative endophthalmitis treated with, Pigmentary retinopathy, 259, 259f 293 Plasma cells, 37, 41 description of, 150 Plasma-­derived proteins, in cornea, 54 sympathetic ophthalmia ­after, 199–200 Plasminogen, 18 uveitis macular edema treated with, 322 Platelet degranulation, 8 Pars planitis Platelet-­activating ­factors (PAF), in inflammation, 12, ancillary tests for, 148–149 20f, 20–21 cataracts in, 151 Platelet-­derived growth ­factors, 24t clinical characteristics of, 147–148 Pneumocystis jirovecii choroiditis, 332, 332f complications of, 151 Polarization, 14 corticosteroids for, 149–150 Polyarteritis nodosa (PAN), 125t, 156–158, 157f description of, 70 Polyarthritis, 143f differential diagnosis of, 148 Polymerase chain reaction (PCR) glaucoma in, 151 for chronic postoperative endophthalmitis, 293 histologic findings in, 148–149 disadvantages of, 90 macular edema in, 147 Goldmann-­Witmer coefficient and, 91 medical management of, 93 syphilis diagnosis using, 225 prognosis for, 149 for Tropheryma whipplei, 245 treatment of, 149–150 in uveitis, 90–91 Pathogen-associated­ molecular patterns (PAMPs), 5 Polymorphonuclear leukocytes (PMNs), 2 Pattern recognition receptors (PRRs) Population-­wide immunity, 63 dendritic cells’ use of, 4 Posner-­Schlossman syndrome, 135, 318 description of, 5 Post-­Ebola virus disease syndrome, 271 toll-­like receptors as, 58 Posterior placoid chorioretinitis, 222, 223f Pediatric uveitis, 320 Posterior scleritis Penetrating keratoplasty B-­scan ultrasonography for, 124 rejection of, 55 clinical pre­sen­ta­tion of, 123 success rate for, 55 optical coherence tomography of, 123f Penicillin G spectral-­domain optical coherence tomography of, for leptospirosis, 233 124–125 for Lyme disease, 231t subtype of, 116t for syphilis, 225–226, 226t vision loss risks, 128 Perimetry, in uveitis evaluations, 90 Posterior segment Perinuclear antinuclear antibody testing, 160 B cells in, 57 Periocular corticosteroids, for uveitis, 95–96, 96–97f Behçet disease manifestations of, 214–215, 215f Peripapillary choroiditis, 201f inflammation of, 83. See also Posterior uveitis Peripheral granuloma, Toxocara, 284f sarcoidosis involvement of, 196–197 Peripheral iridectomy, 318 sympathetic ophthalmia findings in, 200, 201f Peripheral iridotomy, 318 syphilis findings in, 221f, 221–222 Peripheral multifocal choroiditis, 229 T cells in, 57 Peripheral necrotizing retinochoroiditis, 222 toxoplasmosis involvement of, 277 Peripheral retinitis, 251f Posterior sub-­Tenon injection, of periocular Peripheral ulcerative keratitis, 157 corticosteroids, 95–96, 96f, 322 Peripheral uveitis. See Pars planitis Posterior synechiae, in anterior uveitis, 77, 80f Peripheral vascular sheathing, in intermediate uveitis, 75 Posterior synechiolysis, 131f Periphlebitis Posterior uveitis description of, 237 in acute posterior multifocal placoid pigment in multiple sclerosis, 152 epitheliopathy, 169–174, 170–173f Phacoanaphylactic uveitis. See Phacoantigenic uveitis in acute ret­i­nal pigment epitheliitis, 189f, 189–190 in acute zonal occult outer retinopathy, 162–164t Punctate epithelial keratitis, 334f 190–192, 192f Punctate inner choroiditis (PIC) in autoimmune retinopathy, 192–193 characteristics of, 162–164t in birdshot chorioretinopathy, 165–169, 166–168f choroidal neovascularization in, 182 characteristics of, 70, 75 definition of, 181 definition of, 70, 153 fluorescein angiography findings in, 163t, 182, 183f evaluation of, 72–74t indocyanine green angiography findings in, 163t, fluocinolone acetonide implant for, 97–98 182, 183f fundus autofluorescence of, 89 manifestations of, 181–182, 182f idiopathic, 254 optical coherence tomography findings in, 164t, in inflammatory chorioretinopathies of unknown 182, 183f etiology. See Inflammatory chorioretinopathies Punctate keratitis, in reactive arthritis syndrome, 133 in multifocal choroiditis and panuveitis syndrome, Punctate outer ret­i­nal toxoplasmosis (PORT), 279, 279f 179–181, 180f Purified protein derivative (PPD), 28, 238 in polyarteritis nodosa, 156–158, 157f Pyrimethamine, for ocular toxoplasmosis, 281 in punctate inner choroiditis, 181–184, 182–183f signs of, 83 Rapid plasma reagin, 224 in subret­i­nal fibrosis and uveitis syndrome, 184–185 Reactive arthritis syndrome in Susac syndrome, 160f, 160–161 acute anterior uveitis in, 132–133 symptoms of, 76, 77t diagnostic criteria for, 132–133, 133f syphilitic, 224f ­human leukocyte antigen association with, 65t, 132 in systemic lupus erythematosus. See Systemic lupus laboratory tests for, 125t erythematosus Reactive nitrogen products, 16 toxoplasmosis as cause of, 275 Reactive oxygen intermediates, in phagocytosis, 16 Postsurgical necrotizing scleritis, 121, 121f Recurrent uveitis, 76 Prednisone Regional immunity, 51, 52t for Behçet disease, 217 Reiter syndrome. See Reactive arthritis syndrome for uveitis, 102–103 Relapsing polychondritis, 125t Pregnancy, congenital toxoplasmosis in, 275–276, 281 Relative risk, 64 Primary central ner­vous system lymphoma, 303, 304f Relentless placoid chorioretinitis, 173f, 173–174 Primary immune response, 37–38 Reparative macrophages, 16 Primary syphilis, 221 Resting macrophages, 13, 15f Primary vitreoret­i­nal lymphoma Resting neutrophils, 10 age of onset, 303 Ret­ina clinical features and findings of, 304–305, 304–305f acute necrosis of, 250, 251–252f, 330 diagnostic testing for, 305–306 immune responses of, 57–59 fluorescein angiography findings in, 305 immunologic microenvironment of, 57–58 histology of, 306, 307f immunoregulatory systems of, 59 incidence of, 303 metastasis to, 309 Primed macrophages, 14, 15f microglia in, 57 Progressive outer ret­i­nal necrosis (PORN), 250–254, neovascularization of, in uveitis, 324–325 253f, 330 tuberculosis involvement of, 237–238 Proliferative vitreoretinopathy (PVR), 56, 324 Ret­i­nal detachment Propionibacterium acnes, chronic postoperative in cytomegalovirus retinitis, 324, 329 endophthalmitis caused by, 292, 292f rhegmatogenous Prostaglandin analogues, for uveitic glaucoma, 319 chronic peripheral, 311 Prostaglandins risk ­factors for, 151 D2, connective tissue mast cell synthesis of, 3 uveitis as cause of, 324 in uveitic macular edema, 19 Ret­i­nal gene therapy, 58 Protozoal uveitis Ret­i­nal necrosis, 279f cysticercosis, 285–286, 285–286f Ret­i­nal pigment epithelium diffuse unilateral subacute neuroretinitis, 287–288f, acute posterior multifocal placoid pigment 287–289 epitheliopathy involvement of, 171 ocular toxoplasmosis. See Toxoplasmosis, ocular acute zonal occult outer retinopathy involvement in, onchocerciasis, 289f, 289–290 190, 190f toxocariasis, 283–284, 283–285f, 284t birdshot chorioretinopathy findings in, 166 P-­Selectin, 11–12 cytokine synthesis by, 58 Pseudophakic bullous keratopathy, chronic anterior immune responses of, 57–59 uveitis in, 138f immunologic microenvironment of, 52t, 57–58 Psoriatic arthritis immunoregulatory systems of, 59 acute anterior uveitis in, 133 lipofuscin from, 89 clinical pre­sen­ta­tion of, 133, 134f major histocompatibility class II molecule expression laboratory tests for, 125t by, 57 ocular histoplasmosis syndrome findings in, 273 biomicroscopic findings in, 196, 196f peripapillary changes in, 190f chest radiograph findings in, 198 primary vitreoret­i­nal lymphoma findings in, 305 in ­children, 194–195 rubella as cause of, 260 conjunctival involvement by, 195, 195f serpiginous choroiditis findings in, 175 corneal involvement in, 196 subret­i­nal fibrosis in, 178 definition of, 194 toll-­like receptor expression by, 57 diagnosis of, 197–198 Ret­i­nal vascular occlusive disease, in systemic lupus early-­onset, 194, 197 erythematosus, 154, 155f epidemiology of, 194 Ret­i­nal vasculitis etiology of, 195 characteristics of, 75 granulomas associated with, 195, 195f, 197f diseases associated with, 75t histologic findings, 194, 194f ­human leukocyte antigen association with, 65t ­human leukocyte antigen association with, 65t in posterior uveitis, 74t imaging of, 198 vasculopathy versus, 75 laboratory tests for, 125t Retinitis manifestations of, 195–197, 195–197f Chikungunya fever, 268, 268f panuveitis in, 194–199 cytomegalovirus posterior segment involvement in, 196–197 characteristics of, 254–257 ret­i­nal vascular sheathing in, 197, 198f in ­children, 255–256 treatment of, 199 in HIV/AIDS patients, 256, 328–329 Sarcoidosis-­associated uveitis, 148 imaging of, 255f Scavenging macrophages, 13 ret­i­nal detachments secondary to, 324, 329 Schwartz syndrome, 311 treatment of, 256–257 Sclera Epstein-­Barr virus, 257–258, 258f biopsy of, indications for, 91 nonnecrotizing herpetic, 250–254 edema, hyperemia in, 117 syphilitic, 222, 224f immunologic microenvironment of, 52t Retinitis pigmentosa, 192, 310 inflammation of. See Scleritis Retinoblastoma, 308–309 thinning of, in infectious uveitis, 96 Retinochoroidal scar, 278, 278f Scleral grafting, for scleritis, 127, 128f Retinochoroiditis, toxoplasmosis, 278f, 278–280, 297, Scleritis 330f, 330–331 anterior Retinochoroidopathies, 88 diffuse, 116, 118 Retinoic acid-­inducible gene-­I-­like (RIG-­I-­like) nodular, 116, 118–119, 119f receptors, 5 subtype of, 116t Retinopathy classification of, 115–116, 116t autoimmune, 192–193 clinical pre­sen­ta­tion of, 117–123f, 117–124 cancer-­associated, 192–193 diagnosis of, 124–126, 125t HIV-­associated, 327, 328f epidemiology of, 117 measles, 262 episcleritis versus, 117 melanoma-­associated, 192–193 hyperemia in, 117, 117f Rhegmatogenous ret­i­nal detachment immunomodulatory therapy for, 126 chronic peripheral, 311 incidence of, 117 risk ­factors for, 151 infectious uveitis as cause of, 324 actinomycetes as cause of, 119f Rheumatoid arthritis, 125t clinical pre­sen­ta­tion of, 124 Rifabutin, 139 description of, 116 Rifampin, for bartonellosis, 243 microbiological examination for, 125 Rift Valley fever, 265, 265f pathophysiology of, 123–124 Rituximab, for uveitis, 112 treatment of, 127 River blindness. See Onchocerciasis vision loss risks, 128 Roth spots, 294 manifestations of, 115 RPE65 mutation-­associated ret­i­nal dystrophy, 58 methylprednisolone for, 126–127 Rubella, 258–261 necrotizing Rubeola. See Measles anterior, 118f clinical pre­sen­ta­tion of, 118f, 119–122 Sabin’s tetrad, 277 delayed hypersensitivity response in, 116 Sacroiliitis, in reactive arthritis syndrome, 132 description of, 115 S-­antigen, in experimental autoimmune uveoretinitis, diagnosis of, 124 61 granulomatous, 120f, 120–121 Sarcoidosis with inflammation, 120–121, 120–121f angiotensin-­converting enzyme levels in, 198 laboratory tests for, 124 anterior uveitis in, 195–196 mortality rates for, 119 pathophysiology of, 116 Smoking, uveitic macular edema risks associated postsurgical, 121, 121f with, 321 vaso-­occlusive, 120, 121f Snowball opacities vision loss risks, 128 in chronic postoperative endophthalmitis, 292 without inflammation, 121–122, 122f in intermediate uveitis, 82, 147, 148f nodular anterior, 116, 118–119, 119f Snowbanks, in intermediate uveitis, 82, 147 noninfectious, 116 Spectral-­domain optical coherence tomography nonsteroidal anti-­inflammatory drugs for, 126 for multiple evanescent white dot syndrome, 188, pathophysiology of, 116 188f posterior for posterior scleritis, 124–125 B-­scan ultrasonography for, 124 Standardization of Uveitis Nomenclature system, 68–69, clinical pre­sen­ta­tion of, 123 69–70t, 77, 80t, 147, 153 optical coherence tomography of, 123f Staphylococcus aureus, endogenous endophthalmitis spectral-­domain optical coherence tomography of, caused by, 294 124–125 Still disease, 141 subtype of, 116t Stimulated macrophages, 14–16, 15f vision loss risks, 128 Stimulatory hypersensitivity, 38 prognosis for, 127–128 Streptococcus spp, endogenous endophthalmitis caused scleral grafting for, 127, 128f by, 294 surgical treatment of, 127 “String of pearls,” 196, 292, 296f systemic diseases associated with, 115 Stromal interstitial keratitis, 220, 221f topical therapy for, 126 Subacute sclerosing panencephalitis (SSPE), 262–263 treatment of, 126–127 Subconjunctival lymphomas, 308 vision loss risks, 128 Subepithelial keratitis, in reactive arthritis syndrome, Scleromalacia perforans, 121–122, 122f 133 Sclerouveitis, 70 Subret­i­nal fibrosis and uveitis syndrome Secondary immune response, 37–38 characteristics of, 162–164t Secondary syphilis, 221 definition of, 184 Selectins differential diagnosis of, 184 description of, 11 fluorescein angiography findings in, 163t, 184 E-­, 11–12 manifestations of, 184, 184f L-­, 11–12 optical coherence tomography findings in, 164t, 184 P-­, 11–12 treatment of, 185 Self-­tolerance, 34 Subret­i­nal space, immunologic microenvironment of, Sentinel T cells, 5 52t Sentinel vessels, 125 Substance P, 24t Sepsis Substantia propria, conjunctival, 3, 53 bacterial, pathophysiologic responses to, 7 Sub-­Tenon injection, of periocular corticosteroids, lipopolysaccharide in, 7 95–96, 96f, 324 Seronegative spondyloarthropathies Sugiura sign, 206, 207f acute anterior uveitis associated with, 131–134, Sulfadiazine, for ocular toxoplasmosis, 281 133–134f Sunset-­glow fundus, 204, 206, 207f ankylosing spondylitis. See Ankylosing spondylitis Superoxide anion, 16 definition of, 131 Surgery Serous ret­i­nal detachment, tubercular multifocal cataract. See Cataract surgery choroiditis with, 236f for scleritis, 127 Serpiginous choroiditis for uveitis, 113 acute posterior multifocal placoid pigment Susac syndrome, 160f, 160–161 epitheliopathy versus, 171 SYCAMORE study, 100t, 111 characteristics of, 162–164t Sympathetic ophthalmia (SO) course of, 175 anterior segment findings in, 200 definition of, 174 Dalen-­Fuchs nodules in, 200, 202 fluorescein angiography findings in, 163t, 175f description of, 45–46 fundus autofluorescence in, 163t, 176f diagnosis of, 203 imaging of, 174f fluorescein angiography findings in, 200, 201f indocyanine green angiography findings in, 163t, 175 histologic features of, 202 manifestations of, 174–175 ­human leukocyte antigen association with, 65t multifocal, 177 incidence of, 199–200 optical coherence tomography findings in, 164t, indocyanine green angiography findings in, 201, 202f 175, 176f manifestations of, 200–202, 201–202f ret­i­nal pigment epithelium findings in, 175 ocular trauma as cause of, 199 treatment of, 176–177 optical coherence tomography findings in, 201–202, Serpiginous-­like choroiditis, 177, 236, 237f 202f panuveitis in, 199–203 description of, 21 ­after pars plana vitrectomy, 199–200 gamma-­delta, 5 posterior segment findings in, 200, 201f helper. See Helper T cells treatment of, 203 homing of, 38 Vogt-­Koyanagi-­Harada syndrome versus, 203 inhibitors of, for uveitis, 108–1109 Sympathizing eye, 199–200 interleukin-2 synthesis and release by, 31 Synechiae in multifocal choroiditis and panuveitis syndrome, anterior, 318 179 posterior, in anterior uveitis, 77, 80f in posterior segment, 57 Syphilis priming of, 30 acquired, 221 in resting state, 37 anterior segment findings of, 221–222 sentinel, 5 cerebrospinal fluid testing in, 225 subsets of, 34–35, 42 chorioretinitis in, 222–223f tuberculosis response by, 28 congenital, 220, 226 T helper cell-1 definition of, 219 in conjunctiva, 53 diagnosis of, 224–225 delayed hypersensitivity mechanisms mediated by, HIV and, 219–220, 331–332 42, 44t, 53 incidence of, 219 interferon-­gamma produced by, 35 neuro-ophthalmic­ manifestations of, 223–224 macrophage activation by, 42 nontreponemal tests for, 224–225 T helper cell-2 ocular involvement in, 221–224, 222–224f in conjunctiva, 53 penicillin G for, 225–226, 226t cytokines secreted by, 43 polymerase chain reaction diagnosis of, 225 T helper cell-17 posterior segment findings of, 221f, 221–222 in birdshot chorioretinopathy, 34 primary, 221 in delayed hypersensitivity, 44 retinitis in, 222, 224f interleukins produced by, 34 secondary, 221 T lymphocytes. See T cells tertiary, 221 T regulatory cells testing for, 84–85, 148 adaptive, 34 transmission of, 219 in CD4+ T cell development, 43f treatment of, 225–227, 226t description of, 33 treponemal tests for, 224–225 immune responses suppressed by, 35 uveitis caused by, 84–85, 148, 219–227 inducible, 34 Syphilitic choroiditis, 177 naturally occurring, 34 Systemic Immunosuppressive Therapy for Eye Diseases in peripheral tolerance to self-­antigens, 35 Cohort Study (SITE), 100t, 105, 150 Taches de bougie, 196 Systemic lupus erythematosus (SLE) Tachyzoites, 275 antinuclear antibody testing in, 156 Tacrolimus, for uveitis, 108 autoantibodies associated with, 154 Taenia solium, 285 definition of, 153 Takayasu arteritis, 125t description of, 125t T-­cell antigen receptor, in ­human leukocyte antigen–­ lupus choroidopathy in, 154, 155f disease association, 66 lupus retinopathy in, 154, 155f Tertiary syphilis, 221 manifestations of, 154–156 Thiabendazole, for diffuse unilateral subacute pathogenesis of, 153–154 neuroretinitis, 288 ret­i­nal vascular occlusive disease in, 154, 155f Thiopurine-­S-­methyltransferase (TPMT), 106 treatment of, 156 Thrombin, 18 Systemic sarcoidosis. See Sarcoidosis Tissue dendritic cells, 4 Tissue plasminogen activator, 18 T cells Toll-­like receptors (TLRs) in anterior uvea, 56 dendritic cells’ use of, 4 in B cell activation, 35 ­family of, 5 CD4+. See CD4+ T cells lipopolysaccharide, 7 CD8+. See CD8+ T cells ret­i­nal pigment epithelium expression of, 58 clonal expansion of, 37 TLR2, 8 cytotoxic. See Cytotoxic T cells TORCH syndrome, 260 delayed hypersensitivity Touton ­giant cells, 309 description of, 36, 42–44 Toxocara canis, 45, 283, 287 inflammatory diseases mediated by, 44t Toxocara cati, 283 in sympathetic ophthalmia, 45–46 Toxocara granuloma, 45 T helper-­cell 2, 43 Toxocariasis, 283–284, 283–285f, 284t in toxocara granuloma, 45 Toxoplasma gondii, 275–277, 276f, 331 Toxoplasmosis, ocular Tumor necrosis factor-­ in AIDS patients, 331 characteristics of, 23t congenital, 276–277, 277f, 281 immune response suppression by, 25 diagnosis of, 280 inhibitors of Goldmann-­Witmer coefficient for, 91 for Behçet disease, 217–218 punctate outer ret­i­nal, 279, 279f side effects of, 139–140 pyrimethamine for, 281 for uveitis, 105, 110–112, 130–131, 139 retinochoroidal scar caused by, 278, 278f Tumor necrosis factor-­, 23t retinochoroiditis caused by, 278, 278f, 280, 297, 330f, 330–331 Ultrasonography, in uveitis evaluations, 89–90 sulfadiazine for, 281 Undifferentiated anterior uveitis, 146–147 Toxoplasma gondii as cause of, 275–277, 276f, Undifferentiated uveitis, 84 331 Ureaplasma urealyticum, 132 treatment of, 280–282 Uvea Tractional ret­i­nal detachment, 237 anatomy of, 67 Transforming growth ­factor  anterior. See Anterior uvea characteristics of, 24t Uveal lymphoid hyperplasia, 308 in T regulatory cell effector function, 35 Uveal melanocytic proliferation, bilateral diffuse, 309 Treponema pallidum, 219, 224 Uveal melanoma, 308 Triamcinolone acetonide Uveal tract for scleritis, 127 connective tissue mast cells in, 3 for uveitis inflammation of. See Uveitis inferior transseptal injection of, 96, 97f Uveitis intravitreal injection of, 96f, 97 acute posterior sub-­Tenon injection of, 95, 96f anterior. See Anterior uveitis, acute Trimethoprim-­sulfamethoxazole definition of, 75 for ocular toxoplasmosis, 281–282 signs and symptoms of, 71t for Pneumocystis jirovecii choroiditis, 332 ancillary testing for, 84–92, 86–87t, 88–89f for Whipple disease, 245 animal models of, 61–62 Tropheryma whipplei, 243, 311 anterior. See Anterior uveitis Trophozoites, 331 anterior chamber paracentesis for, 90–92 Tuberculin skin test, for tuberculosis, 28, 238 bacterial. See Bacterial uveitis Tuberculoma, 237f blindness caused by, 68 Tuberculosis. See also Mycobacteria tuberculosis in ­children, 320 choroidal involvement in, 236–237f chronic diagnosis of, 238–239 calcific band keratopathy associated with, 313 extrapulmonary, 234 definition of, 75–76 fluorescein angiography findings in, 236, 237f signs and symptoms of, 72t incidence of, 234 systemic corticosteroids for, 102–103 indocyanine green angiography findings in, classification of 236, 237f anatomical, 68–75 isoniazid for, 239–240 clinical features, 75–76 latent, 240 Standardization of Uveitis Nomenclature system, multidrug-­resistant, 239 68–69, 69–70t, 77, 80t, 147 multifocal choroiditis in, 236, 236f clinical features of, 75–76 ocular involvement in, 235–238, 235–238f complications of primary response to, 28 calcific band keratopathy, 143f, 313 pulmonary, 234 cataracts, 313–317 ret­i­nal involvement in, 237–238 choroidal neovascularization, 324–325 risk ­factors for, 234 epiret­i­nal membranes, 323 secondary response to, 28 glaucoma, 317–321 serpiginous-­like choroiditis associated with, 177, 236, hypotony, 321 237f macular edema. See Uveitis, macular edema caused treatment of, 239–240 by tuberculin skin test for, 28, 238 macular hole, 323 uveitis caused by, 234–240, 235–238f ret­i­nal neovascularization, 324–325 Tubulointerstitial nephritis and uveitis (TINU) rhegmatogenous ret­i­nal detachment, 323–324 syndrome vitreous opacification, 323–324 acute anterior uveitis in, 134–135, 135f vitritis, 323–324 CD4+ T cells in, 135 corticosteroids for characteristics of, 134 adverse effects of, 94, 103, 143, 150 diagnostic criteria for, 134 in ­children, 143 ­human leukocyte antigen association with, 65t dose and duration of, 94, 102 intermediate, 149–150 smoking as risk ­factor for, 321 intravitreal, 97–98, 102 treatment of, 322 local, 95 nongranulomatous, 76 nonsteroidal anti-­inflammatory drugs and, 103 ocular hypertension associated with, 317 osteoporosis prevention strategies, 103 pediatric, 320 periocular, 95–96, 96–97f peripheral. See Pars planitis phacoantigenic, 137 phacoantigenic, 136–137 sustained-­release, 95 phacolytic, 136f, 136–137 systemic, 102–103, 150 posterior. See Posterior uveitis tapering of, 94 prevalence of, 68 topical, 94–95, 130 recurrent, 76 definition of, 67 sarcoidosis-­associated, 148 descriptors of, 69t signs of, 77–83, 78t, 79–82f, 80t, 82t diagnosis of Standardization of Uveitis Nomenclature system for, ancillary testing in, 84–92, 86–87t, 88–89f 68–69, 69–70t, 77, 80t, 147 cytology, 91–92 symptoms of, 76, 77t fluid and tissue sampling tests, 90–91 syndromes masquerading as, 84, 303–309. See also history-­taking for, 67, 83–84 Masquerade syndromes imaging for, 67–68, 85–90, 88–89f syphilis testing in, 84–85, 148 laboratory tests, 86–87t treatment of patient ­factors in, 83t, 83–84 carbonic anhydrase inhibitors, 113 physical examination in, 67 corticosteroids. See Uveitis, corticosteroids for signs, 77–83 cycloplegics, 113 differential diagnosis of, 84 goals for, 93 drug-­induced, 139–140f, 139–141 immunomodulatory therapy. See endotoxin-­induced, 8, 62 Immunomodulatory therapy, for uveitis epidemiology of, 68 intravenous immunoglobulin, 113 equine recurrent, 62 mydriatics, 113 evaluation of, 71–74t nonsteroidal anti-­inflammatory drugs, 113, 315 fluid and tissue sampling tests surgery, 113 antibody production, 91 undifferentiated, 84 ocular serology, 91 vision rehabilitation in, 325 polymerase chain reaction tests, 90–91 Uveitis-­glaucoma-­hyphema (UGH) syndrome, 10, in Fuchs uveitis syndrome, 145–146, 146f 137–138 granulomatous, 76 Uveoretinitis, experimental autoimmune, 61–62 HLA-­B27 association with, 64 ­human leukocyte antigen associations in, 63–66 Vaccinations, anterior uveitis caused by, 140 imaging tests for, 67–68, 85–90, 88–89f Valacyclovir color photography, 89 for cytomegalovirus, 256 electroretinography, 90 for herpes simplex virus, 249, 252 enhanced depth imaging optical coherence Vancomycin, for chronic postoperative endophthalmitis, tomography, 85, 88f 293 fluorescein angiography, 88, 89f Varicella-­zoster sine herpete, 248 fundus autofluorescence, 89 Varicella-­zoster virus (VZV) indocyanine green angiography, 89 acute ret­i­nal necrosis caused by, 252 optical coherence tomographic angiography, 87 anterior uveitis associated with, 247–250 optical coherence tomography, 85, 88f infectious scleritis caused by, 124 perimetry, 90 progressive outer ret­i­nal necrosis caused by, 253, 253f ultrasonography, 89–90 skin lesions in, 248, 248f visual field testing, 90 Vascular endothelial growth ­factor (VEGF) inhibitors, immune recovery, 329 310, 322 infectious. See Infectious uveitis for ocular ischemic syndrome, 310 intermediate. See Intermediate uveitis for uveitic macular edema, 322 intraocular lens-­associated, 137–139, 138f Vasculopathy, ret­i­nal vasculitis versus, 75 lens-­associated Vasoactive intestinal peptide, 24t hypopyon in, 6 Vaso-­occlusive necrotizing scleritis, 120, 121f types of, 136f, 136–137 Venereal Disease Research Laboratory (VDRL), 224 macular edema caused by Vernal conjunctivitis, mast cells in, 3 description of, 19, 321–322 Visceral toxocariasis, 283 fluorescein angiography of, 88, 89f Viscocanalostomy, 319 optical coherence tomography of, 85, 88f, 321 Vision loss, scleritis as cause of, 128 in phacoemulsification, 316 Vision rehabilitation, in uveitis, 325 postoperative, 316 Visual field testing, in uveitis, 90 Vitrectomy, diagnostic, 92, 298 HLA-­DR4 and, 204 Vitreoret­i­nal lymphoma ­human leukocyte antigen association with, 65t cytologic studies for, 91 incidence of, 204 primary. See Primary vitreoret­i­nal lymphoma indocyanine green angiography in, 209 Vitreous integumentary changes in, 206 biopsy of lumbar puncture for, 209 indications for, 91–92 manifestations of, 205–207, 205–207f technique for, 92 nivolumab-­induced, 140f collagen type II in, 56 optical coherence tomography findings in, 209, 210f immunologic microenvironment of, 52t, 56 prodromal stage of, 205 opacification in, from uveitis, 323–324 prognosis for, 211 polymerase chain reaction testing of, in uveitis Sugiura sign in, 206, 207f evaluations, 90–91 sunset-­glow fundus in, 204, 206, 207f Vitreous cells sympathetic ophthalmia versus, 203 grading scheme for, 82, 82t treatment of, 211 in intermediate uveitis, 81, 82f ultrasonography of, 209 Vitreous haze, in intermediate uveitis, 81f, 81–82, 82t Voretigene neparvovec-­rzyl, 58 Vitreous hemorrhage, in pars planitis, 151 Voriconazole Vitritis for Candida fungal postoperative endophthalmitis, 298 chorioretinitis with, 72–73t for coccidioidomycosis, 302 description of, 148 Lyme disease in, 229, 229f Wegener granulomatosis, 158 uveitis as cause of, 323–324 Weil disease, 232 Vogt-­Koyanagi-­Harada (VKH) syndrome West Nile virus, 263–264, 264f acute secondary angle-­closure glaucoma associated Whipple disease, 243–245, 244f with, 318 White blood cells. See Leukocytes acute uveitic stage of, 205, 205f White dot syndromes chronic recurrent stage of, 207 acute zonal occult outer retinopathy versus, 192 convalescent stage of, 205–206 definition of, 161 definition of, 204 etiology of, 161, 165 diagnosis of, 206–210, 208t, 209–210f fluorescein angiography of, 88 differential diagnosis of, 210 multiple evanescent. See Multiple evanescent white enhanced depth imaging optical coherence dot syndrome tomography in, 85, 88f Wolbachia, 290 fluorescein angiography findings in, 208–209, 209f histologic findings of, 204 Zika fever, 269–270, 270f

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