Index

A TNF blockers, 62 Adult-onset still’s disease (AOSD) Anterior ischemic optic neuropathy (AION) autoinfl ammatory syndromes, 23 arteritic-AION, 290 etiology infections, 25 fl uorescein angiogram, 304 juvenile rheumatoid (JRA), 23 vasculitic occlusion, 289 nonsteriodal anti-infl ammatory drugs (NSAIDs), 25 Antineutrophil cytoplasmic antibodies (ANCA), serum ferritin level, 26 18, 21, 263–264 Alzheimer’s dementia, 470 Antinuclear antibody assay (ANA), 77, 80, 131–132, 197, 277, 381 American College of (ACR), 1, 131, 132, 146, 163, Antiphospholipid syndrome (APS) 229, 253, 373, 430 antiphospholipid antibodies (aPL) Amyloidosis immune thrombocytopenia (ITP), 176 Alzheimer’s dementia, 470 transverse myelitis, 175 apple-green birefringence, 467 central nervous system manifestations, 174 cardiac involvement, 468 cerebral vasculitis, 177 diagnostic and prognostic feature, 466 classifi cation criteria, 176 Familial Mediterranean fever (FMF), 469 clinical manifestations, 174 fat pad aspiration, 467 cutaneous manifestation (see Livedo reticularis) macroglossia, 468 dermatologic manifestation (see Livedo racemosa) monoclonal gammopathies, 469 menorrhagia, 179 nomenclature and tissue study, 466 nephropathy, 176–177 periorbital , 468 pregnancy management, 179 treatment approaches, 469 thrombosis ANCA-associated vasculitis (AAV) anticoagulation, 178, 179 antithyroid medications, 264 and pregnancy morbidity, 174–175 arthritis and , 261 venous thrombosis, 178 chemosis, 254 Arthralgias, 134 epidemiology, 247 Arthritogenic pathogens, 75 kidneys Arthrocentesis persistent hematuria, 259 bursal fl uid characteristics, 439 renal transplantation, 260, 261 coagulopathy and thrombocytopenia, 437 nasal crusting, 249 glucocorticoids injections, 437, 438 nose irrigating device, 250 Rule of Twos, 437 saddle nose, 247–249 synovial fl uid count, 438 subglottic stenosis, 250–253 Atrophic papillae, 113 treatment and course, 264–267 Autoimmune lymphoproliferative syndrome (ALPS), 164 vasculitic skin lesion, 261 (AS) B anti-TNF therapy, 62 Bath AS disease activity index (BASDAI), 58, 63, 64 C-reactive protein (CRP) levels, 58 B cell lymphomas, 121, 123, 125, 126 Forestier’s disease, 60 Behçet’s syndrome, 284 HLA-B27 positive, 58 vs. autoinfl ammatory disorder, 234 injectable glucocorticoid preparations, 62 azathioprine and interferon-alpha treatment, 238 Klebsiella infections, 61 cardinal characteristic, 233 physiotherapy, 63 clinical manifestations, 238–239 prognosis, 57 colchicine treatment, 233 radiographic pattern, 59 vs. connective tissue disorder, 234 radiological progression, 63 cutaneous hyperpigmentation, 235 sacroiliitis, 59 genital , scrotum, 236, 237 spondyloarthritis, 57 intermittent claudication, 237 syndesmophytes, 60 MRI, 239 systemic infl ammatory disorders, 63 vs. multiple sclerosis, 239

485 486 Index

papulopustular lesions, 234 Colchicine therapy, 37, 366, 370 parenchymal lung disease, 237 Connective tissue disease syndrome, 80, 115, 133 sex differences, disease phenotype, 237 C-reactive protein (CRP), 58, 122, 148 , 233–234 Cryoglobulinemia sterile pustules, 235 clinical manifestations, 271 superfi cial thrombophlebitis, 236 cryocrit correlation, 273 Bell’s palsy, 414 etiologic factor, 274 Benign joint hypermobility syndrome (BJHS), 475 hepatitis C virus (HCV) infections, 271 Bilateral hilar adenopathy, 414–415 mononeuritis multiplex, 273 Birdshot retinochoroidopathy, 385 plasmapheresis, 275 Bone marrow edema syndrome, 483 pulmonary–renal syndrome, 274 Bone scintigraphy, Paget’s disease, 410 renal biopsies, 273 Buerger’s disease. See Thromboangiitis obliterans renal disease, 272 Bullous lupus erythematosus, 139, 140 ribavirin, 274 type II/III cryoglobulinemic vasculitis, 272 C Cutaneous systemic sclerosis, 103 Calcium pyrophosphate dihydrate (CPPD) crystal deposition disease, Cutaneous vasculitis, 116, 228, 280 3, 4 diagnosis D intracellular vs. extracelluar crystals, 377 Dermatomyositis (DM) metabolic causes, 377 amyopathic DM, 195 talc and glucocorticoids crystals, 376 cutaneous fi nding, 194 epidemiology, 377–378 muscle disease, diagnosis, 194 pathophysiology, 378–379 periungual erythema, 195 symptoms and signs vs. polymyositis (PM), 193 clinical clues, 375 skin histopathology, 194 crowned dens syndrome, 373–374 Diffuse idiopathic skeletal hyperostosis (DISH) disease, 60, joints pain, 374 61, 458 medical and surgical procedures, 376 Diplopia, 288 meniscal cartilage of knee and fi brocartilage of wrist, 375 Disc herniation, 455–456, 460 monosodium urate coexistence, 376 Discoid lupus erythematosus (DLE), 144, 145 polyarticular infl amatory arthritis, 373 Disease-modifying antirheumatic drugs (DMARDs), 8, 9, tumoral deposits, 376 69, 75 treatment and outcomes, 379 Distal inter-phalangeal (DIP) joint arthritis, 68 Cannabis arteritis, 323 Dual energy X-ray absorptiometry (DXA), 399 Carpal tunnel syndrome (CTS), 93, 443 Central nervous system (CNS) disease, 120, 147 E Cerebral vein thrombosis, 161 Ehlers-Danlos syndrome (EDS) Cervical myelopathy, 462 bruising, 477 Chrysotherapy, 9 “cigarette paper” scarring and hemosiderin deposition, 474 Churg–Strauss syndrome (CSS) collagen fi bril abnormalities, 471 ANCA-associated vasculitis, 242 collagen studies, 475 capillaritis, 243 EDS-nosology proposal, 472–473 CT scan, lung, 243 emotional support, 477–478 cytotoxic agent treatment, 245 genetic counseling, 475 histopathologic fi ndings, 241 hypermobility type of, 475–476 leukotriene inhibitors, 242 joint protection, 477 lung parenchyma involvement, 243 mitral valve prolapse, 476 lymphadenopathy, 242 skin care, 477 necrotizing and non-necrotizing vasculitis, 242 skin hyperextensibility, 473 nerve infarction, 245 vascular EDS, 477–478 peripheral nerve and myocardium predilection, 244 Enthesitis, 30, 31, 52, 68–69, 74 persistent asthma, 245 Episcleritis, 255 phases of, 241 Erythema elevatum diutinum (EED) rheumatoid nodules, 244 photomicrographs, 332 tongue wasting, 245 skin lesions of, 331 Clinical disease activity index (CDAI), 6, 7 systemic vasculitis, 332 Cogan’s syndrome Esophageal disease, 84 audiograms, 330 Etanercept therapy, 18 autoantibody profi le, 329–330 causing meningitis, 331 F eye in, 331 Familial cold autoinfl ammatory syndrome (FCAS), 41 hearing loss, 329 Familial Mediterranean fever (FMF), 469 interstitial keratitis, 330 cardinal clinical fi ndings, 35 ocular infl ammation, 330–331 colchicine treatment, 36 Cognitive behavioral therapy, FM, 351 MEFV gene mutation, 34 Index 487

renal amyloidosis, 36 coffee consumption, 361 renal transplantation, 37 diabetes associated, 360 septic joint, 34 low-fat dairy product consumption, 362 systemic AA amyloidosis, 37 metabolic syndrome risk, 360 Fibromyalgia (FM) prevalence and incidence of, 359 aerobic exercise vs. nonexercise controls, 351 purine-rich foods, 361 cognitive behavioral therapy, 351 vitamin C intake, 361–362 diagnosis and etiologies, 349 joint erosions with overhanging edges, 357 disease management, 350 joint infl ammation, 358 high glutamate level, 349 synovial fl uid examination, 355, 357 history and physical examination, 347 therapeutic response to colchicines, 355 impact on National Health Service, 348 tophaceous deposits, 356 laboratory evaluation, 347 treatment mood and psychiatric disorders, 345 allopurinol and azathioprine, 367 outcomes in, 350 baby aspirin, 370 sensory stimuli sensitivity, 350 colchicine treatment, 365–366 and sleep disturbance, 347 cyclosporine treatment, 368 specialist care requirement, 349 intensive urate-lowering therapy, 369–370 tender point examinations, 346–347 losartan treatment, 370 Fibro-osseous dysplasia, 45 oral glucocorticoids, 365 Finkelstein’s test, 444 renal and heart transplant recipients, 367–368 Flexor hallucis brevis muscle, 216, 279 serum urate levels, 366–367 Forestier’s disease. See Diffuse idiopathic skeletal hyperostosis tophi in patients, 368 (DISH) disease uric acid in 24hr collection, 356

G H Giant cell arteritis (GCA), 277 Hemophagocytic syndrome, 25 abundant collateral circulation, 303 Henoch-Schönlein purpura (HSP) diagnosis and imaging ankle and dorsum swelling, 226 angiography and ultrasonography, 294 gastrointestinal symptoms, 225 fl uorodeoxyglucose PET (FDG-PET), 295 glucocorticoid treatment, 226 fundoscopy, 290 immunofl uorescence studies, 228 MRI, 294–295 immunopathogenesis, 229 pallid disc edema, 290–291 nonsteroidal anti-infl ammatory agents (NSAIDs), temporal artery biopsy, 296–298 treatment, 226 laboratory testing, 293–294 palpable purpura, 227 methotrexate (MTX), 305 rash of, 225 pathology self-limited disorder, 227 amyloid deposition, 299 skin biopsy, 225 atherosclerosis, 300 Hip resurfacing implant, 485 frozen section analysis, 301 Hormone replacement therapy, osteoporosis, 404 immunohistochemistry, 300 HTLV-I infection, 124 necrosis, 299 Human leukocyte antigen B27 (HLA) screening test, 51 transmural infl ammation, 299 Hypogammaglobulinemia, 420 and PMR, 304 symptoms and signs abnormal temporal arteries, 287–288 I acute unilateral vision loss, 291 Idiopathic infl ammatory myopathies (IIM) anterior ischemic optic neuropathy (AION), 289 azathioprine and methotrexate therapy, 200 audiovestibular dysfunction, 291–292 dermatomyositis (DM) vs. polymyositis (PM), 193 diplopia, 288 immunosuppressive therapy, 200 facial swelling and noises in head, 291 inclusion body myositis (IBM), 200 fever of unknown origin (FUO), 289 MRI, 199–200 jaw claudication, 289 muscle enzymes ocular symptoms, 292 aldolase activity, 196 persistent dry cough, 288–289 creatine kinase (CK) activity, 195 unexplained neck pain, 289 myositis patients, 196 vasculitic neuropathy, 293 serum ALT concentration, 196 visual hallucinations, 291 myositis-specifi c autoantibodies treatment, 302 anti-SRP antibodies, 198 Gastroesophageal refl ux disease (GERD), 84, 85, 171 skin disease, 193–195 Glucocortiosteriod therapy, 8 Idiopathic thrombocytopenic purpura (ITP), 164 Gottron’s , 142, 144, 194, 204, 208 IgG4-associated systemic disease, 116, 313, 314 IgM rheumatoid factor, 28 acute monoarticular arthritis, 355, 357 Immune-mediated inner ear disease (IMIED) epidemiology auditory and vestibular tests, 392–393 488 Index

clinical history, 391 groin area erythema, 224 defi nition neck swelling symptom, 221 sensorineural hearing loss tinnitus and vertigo, 389 and periarteritis nodosa, 224 sympathetic otitis, 389–390 peribronchial infi ltrates and mild interstitial infi ltrates, 223 temporal bone and audiovestibular apparatus, 390 periodic echocardiograms, 222 differential diagnosis, 393–395 RSV or PIV test, 222 treatment, 395 Koebner phenomenon, 142 vestibular nystagmus, 391–392 Immune thrombocytopenia (ITP), 176 L Inclusion body myositis (IBM), 200 Legg-Calve-Perthes disease, 433 Infl ammatory arthritis, 69, 92, 244, 273, 373 Leukocytoclastic vasculitis, 18, 225, 261, 332, 333 Infl ammatory bowel disease (IBD), 54, 58, 126 Leukopenia, 122, 163, 283 Infl ammatory eye disease Livedo racemosa, 176, 177, 282, 283 birdshot retinochoroidopathy, 385 Livedo reticularis, 176, 177, 227, 282, 283 CMV vasculitis, 382 Löfgren's syndrome, 417 cotton wool spot diagnosis, 386 Low back and neck pain episcleritis, 382, 385–386 cervical myelopathy, 462 glucocorticoids treatment, 382–383 degenerative spondylolisthesis, 457–458 infectious uveitis, 383–384 disc herniation, 455–456 intraocular CNS lymphoma, 383 evaluation and treatment, 458–459 ocular , 387 history and physical examination, 452–454 orbital pseudotumors, 384 imaging pars planitis, 385 MRI and CT scan, 455 retinal vasculitis, 381 red fl ags, 454 Roth spots, 387 important causes, 458 scleritis diagnosis, 381–382 mechanical cause, 452, 460 tubulointerstitial nephritis with uveitis (TINU), 386 spinal stenosis, 456–457 Infl ammatory myopathies, 197–198 spine basic anatomy, 451 surgery, 459–460 J whiplash injury, 461 Jaccoud’s , 3, 117, 136 Lupus anticoagulant (LA) phenomenon, 174 Juvenile dermatomyositis (JDM) Lupus pernio, 416 acanthosis nigricans, 208 Lupus psychosis, 147 bone health, 209 Lyme disease, 103 calcinosis, 206 clinical features, 204 M disease control, 209 Macrophage activation syndrome (MAS), 28, 163 dystrophic calcifi cations, 206–207 MALT lymphoma, 116, 123, 125 Gottron’s , 204 Membranoproliferative glomerulonephritis (MPGN), 273 heliotrope discoloration and erythema, 204 Meniere’s disease, 394 IIM differential diagnosis, 205 Metacarpophalangeal (MCP) joints, 3, 80, 82, 425 metabolic abnormalities, 207 Mevalonic aciduria, 39 muscle biopsy, 203 Microscopic polyangiitis (MPA), 283 oral glucocorticoid therapy, 208–209 alveolar hemorrhage, 256–257 overlap syndrome, 210 clinical phenotypes, 256 periungual nailfold capillary change, 206 interstitial fi brosis, 255 photosensitive rash, 206 pulmonary hemorrhage, 256 signs and symptoms, 208 RBC with glomerulonephritis, 259 sun protection, 209 treatment and course, 264–267 Juvenile idiopathic arthritis (JIA), 27–31, 51 vasculitic neuropathy, 262 Juvenile (JRA), 23 Mikulicz disease, 124–125 Juvenile spondyloarthropathy Mixed connective tissue disease (MCTD) enthesitis clinical feature, 52 anti-RNP antibody emergence, 170 enthesitis-related arthritis (ERA), 51 diffuse swelling, fi ngers, 171 infl ammatory bowel disease (IBD), 54 gastroesophageal refl ux, 171 infl ammatory enthesitis, 53 myositis, 171 Osgood–Schlatter’s disease, 52 nasal septal perforation, 172 Patrick/FABER test, 54 sclerodactyly and digital ischemia, 171 sacroiliitis, 53 sicca symptoms and pulmonary hypertension, 170 tumor necrosis factor (TNF) inhibitors, 55 unilateral or bilateral trigeminal neuropathy, 169 Monogenic autoinfl ammatory syndromes K cherubism, 45–46 Kawasaki’s disease chronic recurrent multifocal osteomyelitis (CRMO) classifi cation criteria, 224 congenital dyserythropoietic anemia (CDA), 43 conjunctival injection, 222 primary intraosseous lymphoma, 44 coronary arteries dilation, 223 cryopyrin-associated periodic syndromes (CAPS) Index 489

bony deformities, 42 localized scleroderma, 103 CIAS1 mutation, 41 morphea, 103–104 cochlear infl ammation, 42 nephrogenic fi brosing dermopathy (NFD), 97 familial Mediterranean fever (FMF) risk factor, 100 cardinal clinical fi ndings, 35 scleromyxedema, 104–105 colchicine treatment, 36 therapeutic agents, 102 MEFV gene mutation, 34 yellow scleral plaques, 101, 102 renal amyloidosis, 36 Neutropenia, 122 renal transplantation, 37 Nonsteroid antiinfl ammatory drugs (NSAIDs), 53, 61 septic joint, 34 systemic AA amyloidosis, 37 O hereditary periodic fever syndromes, 33 Oral glucocorticoid therapy, 208–209 hyper immunoglobulin D and periodic fever syndrome (OA) hyper IgD syndrome (HIDS), 39 aging outcome, 425 prognosis, 40 articular cartilage damage, 426 splenomegaly and gastrointestinal manifestations, 39 carpometacarpal joint, 433 PAPA syndrome, 44–45 dimethylsulfoxide treatment, 427 tumor necrosis factor receptor-associated periodic syndrome glucocorticoid injections, 429 (TRAPS) groin pain, 431–432 anti-IL-1 treatment, 38 joint degeneration TNFRFS1A mutations, 39 course, 428 Muckle-Wells syndrome (MWS), 23, 41 initial stages, 430–431 Multicentric reticulohistiocytosis, 5 joint pain, 426 Muscle , 30, 205, 440 knuckle cracking, 425 Musculoskeleton complaints Legg-Calve-Perthes disease, 433 ankle, 448–449 leg length discrepancy, 428 coccyx, 445 mild hip dysplasia, 431, 432 elbow pain MRI, 428 antecubital area swelling, 443 patellofemoral OA, 431 septic olecranon bursitis, 442 radiographs assessment, 429, 430 hand, 444–445 recreational running risk, 425 hip, 445–446 risk factors, 428 knee slipped capital epiphysis, 432 anserine bursa, 448 viscosupplementation effi cacy, 433 aspiration to, 447 Osteonecrosis best injecting way, 446 antero-posterior radiograph, stage IV, 485 leg cramps, 445 arthroplasty treatment, 485 shoulder pain bone marrow edema syndrome, 483 brachial plexopathy, 440 early stages, 481–482 humeral head superior migration, 440 femoral head microcrystalline disease, 441 blood supply disruption, 482 palpable effusion, 442 core decompression, 484 posterolateral approach, injections, 441 hip resurfacing, for collapse of, 485 subacromial bursa injections, 440 hip MRI, 482–483 wrist plain radiograph, 482 carpal tunnel syndrome (CTS), 443 risk factors, 481 Finkelstein’s test, 444 secondary osteonecrosis Mycophenolate mofetil (MMF), 152, 155, 186, 367 vs. spontaneous, 486 Myocardial infarction, 16, 134, 151 treatment algorithm, 483 Osteonecrosis of the jaw (ONJ), 404–405 N Osteoporosis Neisseria gonorrhea, 76 diagnosis, bone mass measurement Neonatal lupus erythematosus (NLE), 162 DXA scans, 399 Neonatal onset multisystem infl ammatory disorder (NOMID) fracture risks, 399 syndrome, 41, 42 low bone density, 399–400 Nephrocalcinosis, 119 epidemiology Nephrogenic systemic fi brosis (NSF) and scleroderma mimickers among African Americans, 397 clinicopathological diagnosis, 99 among men, 398 dermal tissue injury, 102 hereditary disorder of collagen, 398 Doppler techniques, 101 low trauma fractures, 398–399 eosinophilic fasciitis, 105 prevention fi brosing disorders, 98 calcium and sodium effects, 400–402 gadolinium-based contrast agent, 100 vitamin D, 402–403 hemodialysis, 103 treatment histopathological fi ndings, 99 bisphosphonates, 404–405 kidney dysfunction, 100 general considerations, 403–404 490 Index

hormone replacement therapy, 404 and GCA, 304 treatment WHO defi nition, 397 prednisone treatment, 302, 304 vs. RA, 304 P shoulder pain symptom, 288 Paget’s disease of bone Polymyositis (PM) asymptomatic disorder, 409 vs. dermatomyositis (DM), 193 etidronate and teraparatide treatment, 411 vs. IBM, 199–200 monostotic and polyostotic disorder, 410 immunosuppressive therapy, 200 radiographic fi ndings, 411 Posterior reversible encephalopathy syndrome (PRES), 147 Parainfl uenza virus (PIV) test, 222 Prednisone monotherapy, 313 Pars planitis, 384, 385, 419 Pregnancy, rheumatic diesease Pauciarticular JIA, 29, 31 antiphospholipid syndrome (APS), 189 Pauci-immune glomerulonephritis, 20 breastfeeding, 187 Pediatric systemic lupus erythematosus dyspnea, 184 autoimmune lymphoproliferative syndrome (ALPS), 164–165 elevated erythrocyte sedimentation rate (ESR), 184 cerebral vein thrombosis, 161 infertility, 183 cutaneous neonatal lupus, 162, 163 medications discoid lesions, 165 hydroxychloroquine, 186 glucocorticoids dose, 161 ibuprofen, 187 herpes virus infection, 163 methotrexate or lefl unomide, 186 MAS and macrocephaly, 163 mycophenolate mofetil (MMF) and azathioprine, 186 neonatal lupus erythematosus (NLE), 162 steroid, 185 proliferative lupus nephritis, 164 sulfasalazine, 187 , 165 neonatal lupus syndromes, 189–190 Pediatric vasculitis ovarian stimulation, 184 classifi cation scheme, 231 proteinuria and creatinine level, 184 Henoch-Schönlein purpura (HSP) rheumatoid arthritis, 190 gastrointestinal symptoms, 225 systemic lupus erythematosus (SLE) glucocorticoid treatment, 226 lupus fl are and preeclampsia, 188 immunofl uorescence studies, 228 oral contraceptives, 187 immunopathogenesis, 229 renal allografts, 188 nonsteroidal anti-infl ammatory agents (NSAIDs), treatment, thrombocytopenia, 188 226 Primary angiitis of the CNS (PACNS) palpable purpura, 227 brain imaging, 316 rash of, 225 lumbar puncture, diagnosis, 315 self-limited disorder, 227 with mass lesion, 315 skin biopsy, 225 vs. RCVS Kawasaki’s disease, 221–224 angiographic features, 317 polyarteritis nodosa (PAN), 229–231 blood and CSF test results, 319 von Willebrand factor antigen, 231 clinical features, 317 Peritoneal dialysis, 103 cross-sectional neuroimaging, 317–318 Plantar fasciitis, 448, 449 Primary biliary cirrhosis (PBC) symptom, 83 Pneumocystis carinii (jiroveci) pneumonia (PCP), 150 Proliferative lupus nephritis, 164 Polyarteritis nodosa (PAN) (PsA), 29 ACR classifi cation criteria, 229 coronary artery disease, 70 antinuclear antibody (ANA), 277 cutaneous psoriasis, 69 atrophie blanche, 285 dactylitis, 68 cutaneous PAN, 230 infl ammatory arthritis, 69 diabetes mellitus, 278 vs. RA, 68 diagnosis, 279 rheumatoid arthritis (RA), 67 hepatitis B-related PAN, 229 skin and joint disease, 70 leg ulcers diagnosis, 281 Pulmonary arterial hypertension (PAH), 77, 88–90 livedoid vasculopathy, 285 Pulmonary fi brosis, 86 livedo racemosa, 283 livedo reticularis, 282 R magnetic resonance angiography, 284 Raynaud’s phenomenon (RP), 117, 123, 172, 324 microaneurysm, 283, 284 acro-osteolysis, 79 nodular vasculitis, 282 antinuclear antibody assay (ANA), 77 peripheral nervous system vasculitis, 278 calcium channel blockers, 78 renal vasculature, 284 cyanosis ischemia correlation, 79 skin biopsy, 285 dilated capillary loops, 78 skin ulcer diagnosis, 282 skin induration, 77 thrombocytopenia, 283 vasodilator therapy, 79 vasculitic neuropathy, 278, 279 (ReA) visceral abdominal angiography, 284 circinate balanitis, 76 Polymyalgia rheumatica (PMR) dactylitis pattern, 73 Index 491

diagnostic test, 74 Rheumatoid vasculitis disease-modifying antirheumatic drugs (DMARDs), 75 anti-TNF therapy, 17 enthesitis, 74 corneal melt syndrome, 20 gastrointestinal infections, 75 cyclophosphamide, 15 gonococcal arthritis, 76 glomerular pathology, 21 , 73 leg ulcers, 17 psoriatic arthritis, 76 mononeuritis multiplex symptom, 18 rheumatic fever, 74 myocardial infarction, 16 rheumatoid arthritis, 75 pulmonary rheumatoid nodules, 20 , 73 pyoderma gangrenosum, 17 synovial fl uid, 74 rheumatoid factor, 21 Relapsing polychondritis (RP) symmetric polyneuropathy, 18 cartilaginous part infl ammation, 339 vasculitic neuropathy diagnosis, 19 chest radiograph, 341 Rodnan skin score method, 84 direct laryngoscopy, 342 Roth spots, 387 irreversible hearing loss, 339 nose bridge collapse, 341 S patient with traschestomy, 342 SAPHO syndrome, 44 pinna involvement, 340 Sarcoidosis recurrent pneumonias or bronchiectasis, 342 BAL, CD4:CD8, 415 respiratory symptoms, 340 Bell’s palsy, 414 subglottic stenosis tomogram, 341 bilateral hilar adenopathy, 414–415 vertigo and hearing diffi culties, 339 in black patients, 413 Renal dysfunction, 274 clinical outcomes, 414, 422 Respiratory syncytial virus (RSV) test, 222 ECG monitoring, 419 Reversible cerebral vasoconstriction syndromes (RCVS) erythema nodosum, leg, 417 bad migraine attack, 319 Fatigue Assessment Scale (FAS), 418 brain imaging, 318 granulomatous disease, 420 key diagnosis elements, 317 high resolution CT scan, 415 vs. PACNS liver involvement, 418 angiographic features, 317 Löfgren’s syndrome, 417 blood and CSF test results, 319 lupus pernio, 416 clinical features, 317 pars planitis and optic neuritis, 419 cross-sectional neuroimaging, 317–318 peribronchial thickening, 416 pharmacological blood pressure observation, 320 predictive features, 417 transcranial Doppler studies, 319 prevalence of, 414 vasoconstrictive medications avoidance, 320 pulmonary hypertension incidence, 422 Rheumatoid arthritis (RA), 107 pulmonary sarcoidosis, 420–421 adverse effects traction bronchiectasis, 416 cardiovascular disease, 11 Seronegative , 67, 69, 452 hepatitis B virus infection, 10 Seropositive rheumatoid arthritis, 78 clinical features Serum monoclonal gammopathy, 124 amyloidosis, 3 Sjögren’s syndrome (SjS), 81, 162 synovitis and radiographic progression, 2 classifi cation and epidemiology diagnosis, 1 American–European classifi cation criteria, 108 disease assessment Klinefelter syndrome, 108–109 patient global assessment (PGA), 7 patient classifi cation/diagnosis, 107 simplifi ed disease activity index, 6 differential diagnosis, 124–125 immunizations, 11 extraglandular involvement mimickers Devic’s disease, 120 calcium pyrophosphate dehydrate (CPPD) deposition disease, dyspareunia, 122 3, 4 dysphagia, 116 chronictophaceous gout, 3 erosive symmetrical arthritis, 117 erosive osteoarthritis, 5 glomerulonephritis, 119 parvovirus infection, 4 Hepatitis C virus (HCV) infection, 118 symmetrical , 3 interstitial fi brosis, 117 serological features and radiology, 5–6 lacrimal/parotid glands, 121 serum rheumatoid factor, 6 liver function tests, 118 treatment lymphocytic vasculitis, 116 antibacterial effects, 9 pancreatic cancer, 119 biologic DMARDs, 7 peripheral neuropathies, 119–120 cholestyramine, 8 sensorineural hearing loss, 121 human antichimeric antibodies [HACA], 10 sensory neuropathy, 120 methotrexate (MTX) therapy, 8, 9 thyroid disease, 122 tumor necrosis, 9 trigeminal neuralgia, 120 Rheumatoid nodules, 2 immunological assays 492 Index

hematological neoplasm, 124 proliferative lupus nephritis, 164 Raynaud’s phenomenon, 123 treatment keratoconjunctivitis sicca, 107 antimalarial therapy, 152 laboratory fi ndings, 122–123 azathioprine, 155 parotid and submandibular involvement, 115–116 chloroquine treatment, 153 pathogenesis, 109 immunosuppressive drug regimens, 152 prognosis, 125–126 osteonecrosis, 154 sicca Features quinacrine therapy, 153 ocular manifestations, 109–111 renal disease, 153–154 oral manifestations, 111–115 Varicella zoster immunization, 150 systemic treatment, 126 Systemic-onset juvenile idiopathic arthritis (sJIA) SLE disease activity index (SLEDAI), 146 antiCCP antibodies, 28 Spinal stenosis, 456–457 knee monoarthritis, 30 Spondylolisthesis, 457–458 leukemic arthritis, 31 Subacute cutaneous lupus erythematosus oligoarticular JIA, 29 (SCLE), 138, 144 pauciarticular JIA, 31 Sydenham’s chorea, 161 sacroiliitis, 30 Synovial entheseal complex (SEC), 69 thrombocytosis, 31 Systemic lupus erythematosus (SLE) Systemic rheumatic diseases, 133 arthritis of, 163 Systemic sclerosis (scleroderma) autoimmune lymph proliferative syndrome antibodies and disease phenotypes (ALPS), 164 anti-centromere antibodies, 80 central nervous system lupus epidemiologic risk factor, 82 neuropsychiatric lupus, 146 the health assessment questionnaire disability index (HAQ-DI), optic neuritis, 147 82 cerebral vein thrombosis, 161 pulmonary fi brosis, 83 clinical features RNA polymerase, 81 autoimmune hemolytic anemia (AIHA), 136 cardiac disease, 90 autoimmune liver disease, 134 diagnosis, 80 autoimmune vs. lupus-associated hepatitis, 135 gastrointestinal involvement cardiovascular disease, 133 gastric antral vascular ectasia, 84 idiopathic infl ammatory myopathies, 136 proton pump inhibitors (PPIs), 85 primary biliary cirrhosis, 135 xerostomia, 84 cutaneous lupus interstitial lung disease (ILD) alopecia areata, 140 bronchoalveolar lavage (BAL), 86 clinical effects, 138 cyclophosphamide therapy, 87 discoid lupus lesions, 144 forced vital capacity (FVC), 85 epidermolysis bullosa acquisita, 139 glucocorticoids, 86 eruptive dermatofi bromas, 141 velcro crackles, 88 koebnerize, 142 Wegener’s granulomatosis etanercept trial lupus panniculitis, 140 (WGET), 88 malar rash, 136 musculoskeletal manifestations, 91–92 mycobacterial infections, 141 peripheral neuropathy, 92 nasolabial folds, 137 pulmonary arterial hypertension (PAH) oral ulcerations, 142 echocardiography, 88 vermillion border, 143 parenchymal lung disease, 89 vitamin D levels, 145 pulmonary artery pressure, 90 diagnosis Raynaud’s phenomenon (RP) and digital ischemia American College of Rheumatology acro-osteolysis, 79 (ACR), 131, 132 antinuclear antibody assay (ANA), 77 autoimmune thyroiditis, 132 calcium channel blockers, 78 skin biopsy, 133 cyanosis ischemia correlation, 79 diffuse central nervous system dysfuntion, 164 dilated capillary loops, 78 diffuse/focal myocarditis, 151 skin induration, 77 discoid lesions, 165 vasodilator therapy, 79 erythrocyte sedimentation rate (ESR), 148 scleroderma renal crisis idiopathic thrombocytopenic purpura (ITP), 164 ACE inhibitor, 90 lupus nephritis angiotensin II inhibitors, 91 glomerulonephritis, 146 skin disease renal dysfunction, 145–146 pterygium inversum unguis condition, 83 serum creatinine, 145 scleroderma, 84 partial thromboplastin time (PTT), 161 telangiectasias, 83 pregnancy lupus fl are and preeclampsia, 188 T oral contraceptives, 187 Takayasu’s arteritis (TA) renal allografts, 188 abnormal angiogram, 312 thrombocytopenia, 188 cardiomyopathy differential diagnosis, 310 Index 493

carotid bypass grafts, CNS symptoms, 313 electrodiagnosis and biopsy contraindicated pregnancy, 314 bilateral nerve conduction studies, 216 ESR and CRP gauges, 312 fl exor hallucis brevis muscle examination, 216–217 femoral artery involvement, 310 muscle biopsy, 217–218 fi bromuscular dysplasia, 311 multiple mononeuropathies, 216 vs. GCA, 311 neurological pattern, 213 IgG4-related systemic disease, 313, 314 neurophysiologic patterns, 217 markedly abnormal acute phase response, 312 pain and paresthesias, 215 monophasic illness, 310 pathophysiology prednisone monotherapy, 313 nerve fi ber pathology, 218 Temporal artery biopsy, GCA neural vasculature, 218 contraindications to, 296 perivascular infl ammatory infi ltrates, 218–219 frozen section analysis, 301 vascular histopathology diagnosis, 219 GCA diagnosis, 296 polyneuropathy immunohistochemistry aid, 300 manifestations, 213 likelihood estimation ratio, 288 onset and progression, 214 longitudinal section of, 299 sensory defi cits, 214 optimal and risky sites, 298 systemic and nonsystemic vasculitis, 215 in scalp, 298 Vestibular nystagmus, 391–392 Tendinitis, 439 Thromboangiitis obliterans W amputation risk, 321 Wegener’s granulomatosis (WG), 10 clinical and angiographic fi ndings, 322, 326 bladder, 261 early lesions in, 322 clinical features, 248 extra-extremity disease, 325 clinical phenotypes, 256 necrotic fi ngertips amputation, 325–327 CNS, 262–263 nicotine addiction, 321 consensus classifi cation criteria, 230 Raynaud’s phenomenon, 324 eye splinter hemorrhages, 325 episcleritis, 255 superfi cial thrombophlebitis manifestation, 323–324 orbital pseudotumor, 254 systemic vasculitis, 322 tarsal structure in, 254 young men disease, 323 kidneys Tubulointerstitial disease, 119 granulomas in renal biopsy, 258 Tubulointerstitial nephritis with uveitis (TINU), 386 prednisone treatment, 260 prognostication, 260 U lungs Undifferentiated connective tissue disease (UCTD), 169. See also chest radiography, 257 Mixed connective tissue disease (MCTD) normal saline nebulizer treatment, 257 U3RNP autoantibodies, 80 venous thrombotic events (VTE), 255–256 Urticarial vasculitis misdiagnosis, 250 hypocomplementemic vasculitis mouth low complement C1q level, 335 roof erosion, 254 vs. normocomplementemic, 333 strawberry gums, 253 palpable purpura, 336 tongue ulcer, 253 scleritis, 337 nasal crusting, 249 lesions of 3 day’s duration, 335 peripheral seventh cranial nerve palsy, 262 prednisone treatment, 334 splenic involvement, 263 skin biopsy, 334 subglottic stenosis, 229 Uveitis-band keratopathy, 29 diagnosis over phone, 251 dyspnea on exertion, 250 V fl ow volume loops, 252 Vasculitic neuropathy metal tracheal stents avoidance, 251 cyclophosphamide and glucocorticoids treatment and course, 264–267 treatment, 219–220 wrist arthritis, 262 diagnosis, 19 Whiplash injury, 461