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Overview of Episcleritis Conjunctivitis vs Episcleritis and Scleritis Conjunctivitis is common. Episcleritis and Scleritis are not. Thomas F. Freddo, O.D., Ph.D. Apply topical anesthetic. Then use a cotton PPfrofessor an d Former DitDirector swabbt to move th e conjjtilunctival surffhilace while School of Optometry observing through slitslit--lamp.lamp. If the dilated and congested vessels move with the swab, its University of Waterloo conjunctivitis. Advice on holding lid open -roll- roll--upup awning
Episcleritis DDX Sectorial Redness
A relatively benign, Staph marginal recurrent disease rarely associated with ulcer systemic disease. Limbal Visual acuity is normal phlyc tenu le Mild, occasional pain Limbal More often in women than men, as is malignancy generally true for most Pterygium autoimmuneautoimmune--typetype diseases Two varieties: simple & Simple Episcleritis nodular
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EPISCLERITIS Simple Episcleritis
Often recur at 1- to 3-month intervals. Episodes appear acutely Most cases are idiopathic occurring more often and usually last 7-10 days in women than men and usually in their 30’s or Most resolve after 2-3 40’s. weeks. Prolonged episodes may be A preliminary workwork--upup is worth considering only more common in patients if your directed case history leads to suspicion with associated systemic of an underlying systemic association. conditions. Systemic associations found primarily through Some patients note that history in 1/3 of patients. Labs are to confirm. episodes are more common No correlation between type, laterality or in the spring or fall. recurrences and presence of associated Precipitating factor is rarely systemic disease found.
EPISCLERITIS Nodular Episcleritis Differential Diagnosis
Takes longer Virtually any infectious disease can be to resolve (4-6 associated with episcleritis – CBC with differential , looking for leukocytosis/leukocytosis/eosinophiliaeosinophilia weeks) Bacteria, including tuberculosis, Lyme disease, and syphilis Viruses, including herpes Fungi Parasites Other more common causes – Atopy - rule out by history and skin appearance Foreign Body – rule out by history and exam Normal tears = 7.1 Chemicals – rule out by history, measuring tear pH and exam Gout Adult Rheumatoid Arthritis Seronegative spondyloparthropathies
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If the patient is unresponsive to treatment a referral EPISCLERITIS to an cornea/external disease specialist is prudent in order to explore the more rare causes including: Differential Diagnosis
Other rare causes/associations Systemic Lupus erythematosus Polyarteritis nodosa Gout TT--cellcell leukemia Paraproteinemia Adult Rheumatoid arthritis Paraneoplastic syndromes - Sweet syndrome, dermatomyositis WiskottWiskott--AldrichAldrich syndrome Seronegative spondyloarthropathies Adrenal cortical insufficiency Necrobiotic xanthogranuloma Progressive hemifacial atrophy Following transscleral fixation of posterior chamber intraocular lens Insect bite granuloma Malpositioned Jones tube
EPISCLERITIS Differential Diagnosis
Differential Diagnosis AUTOIMMUNE DISEASES GoutGout–– acute attacks of foot or lower joint pain especially in great toe but any synovial joint can be involved. Elevated uric acid level as a result of a Adult rheumatoid arthritis defect in purine metabolism. Results in deposition of crystallized uric acid as “tophi”. R/O in history or exam . Get urine sample for uric acid level . In acute attacks white count elevated with left shift and sed rate (ESR) may be Seronegative spondyloarthropathies elevated. Diet. MedicationMedication of choice allopurinolallopurinol.. Ankylosing spondylitis Inflammatory bowel disease Psoriatic arthritis Reactive arthritis (Reiter’s syndrome)
Removal of tophi
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If the patient is unresponsive to treatment a referral to an Episcleritis/Scleritis and relationship with the cornea/external disease specialist is prudent in order to seronegative spondyloarthropathies explore the more rare causes including:
Read and review: Other rare causes/associations Systemic Lupus erythematosus Polyarteritis nodosa www.arc.org.uk/about_arth/med_report TT--cellcell leukemia s/series4/tr/6604/6604.htm Paraproteinemia Paraneoplastic syndromes - Sweet syndrome, dermatomyositis WiskottWiskott--AldrichAldrich syndrome Adrenal cortical insufficiency www.orthop.washington.edu/uw/tabID_ Necrobiotic xanthogranuloma Progressive hemifacial atrophy 3376/print_full/ItemID_130/mid_0/Arti Following transscleral fixation of posterior chamber intraocular lens cles/Default.aspx Insect bite granuloma Malpositioned Jones tube
Episcleritis – Work-up Treatment
Ocular therapy Useful laboratory studies in this Simple episcleritis often requires no treatment. Cold compresses and iced artificial tears are useful for patients with mild-to-moderate symptoms. Patients group of patients include: with severe or prolonged episodes may require topical corticosteroids but serum uric acid - Gout steroids have been shown to worsen recurrences. complete blood count with Nodular episcleritis is more indolent and may require local corticosteroid drops or anti-inflammatory agents. differential Topical pred phosphate penetrates the eye less well and thus may be preferred rheumatoid factor over acetate in these cases to mitigate against steroid response. erythrocyte sedimentation rate Systemic therapy If nodular episcleritis is unresponsive to topical therapy, systemic anti- Venereal Disease Research inflammatory agents may be useful. Laboratory (VDRL) test Flurbiprofen (100 mg tid) is usually effective until inflammation is suppressed. fluorescent treponemal antibody If there is no response to flurbiprofen, indomethacin should be used; 100 mg absorption (FTA-ABS) daily and decreased to 75 mg when there is a response. chest x-ray Many patients who do not respond to one nonsteroidal anti-inflammatory agent (NSAID) may respond to another NSAID. Activity Sunglasses may be useful for patients with sensitivity to light.
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Episcleritis vs. Scleritis Pain in the Differential
View patient in MOST Important - judge DDx: Severe ocular daylight and/or red- the pain. If it is deep pain: free. penetrating pain, Corneal surface radiating all around the Episcleritis will look problems red, scleritis will head and/or awakens the Angle closure have a deeper, patient from sleep, think glaucoma purple-red scleritis. OTC analgesics coloration not well will not lessen pain. Scleritis, especially seen by slit-lamp. If pain worsens with eye with anterior uveitis. movement, think scleritis.
Note deep purple-red background
EPISCLERITIS VS SCLERITIS: Use 2.5% phenylephrinephenylephrine.. Generally, it will whiten an episcleritisepiscleritis,, not a deep scleritis Scleritis
Most common 4th4th--6th6th decade Episcleritis In 15% of cases, scleritis is the presenting sign of collagen vascular disease. More in females Scleritis 1.6:1 OneOne--thirdthird are recurrent
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Scleritis Anterior Scleritis
Rheumatoid arthritis is the underlying disease for approximately one sixth of patients suffering from scleritis Anterior Other connective tissue and autoimmune diseases seen with Diffuse - most scleritis include the following: common Systemic lupus erythematosus(SLE) Nodular Polyarteritis nodosa Necrotizing Seronegative spondyloarthropathies Ankylosing spondylitis Most cases stay as Psoriatic arthritis same type as at Reactive arthritis original Inflammatory bowel disease presentation, but Sarcoidosis some advance Tuberculosis Syphilis Lyme *List is almost identical to anterior uveitis
Treatable Masquerades Diffuse Anterior Scleritis
Most common form Infectious scleritis is a possibility that should be Insidious onset over 5-10 evaluated. days Herpes scleritis can present as a masquerade of Easily misdiagnosed as scleritis and should always be considered if the episcleritis patient has unilateral scleritis. Increased scleral Syphilis is another important masquerader that translucency when needs to be ruled out, as it is treatable. (Luetic healed serology) Least associated with Conditions such as severe rosacea or severe systemic disease atopy or allergy can also induce scleritis, so a thorough patient history is essential.
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Necrotizing Anterior Nodular Scleritis Scleritis
Nodule firm May occur and immobile with or Onset insidious without iflinflamma tion
Necrotizing Scleritis Wegener’s Granulomatosis
INFLAMMATORY NON-INFLAMMATORY Usually begins with respiratory symptoms Runny nose, with puspus--filledfilled discharge Nosebleeds Sinus pain and inflammation (sinusitis) Most severe and Scleromalacia perforans Hoarse voice destructive form Painless melting of sclera 29% di e withi n 5 yrs of Inflammation of the gums leaving choroid exposed Ear infections onset to conjunctiva 50-90% have underlying Cough disease, usually Spontaneous perforation Chest pain rheumatoid arthritis, rare Coughing up blood ((hemoptysishemoptysis)) Wegener’s, relapsing Most often with Shortness of breath polychondritis rheumatoid arthritis Wheezing General ill feeling (malaise) But can present with scleritisor granulomatousanterior uveitis
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Work up of Wegener’s ANCA
p-ANCA, or protoplasmic- AntiAnti--neutrophilneutrophilcytoplasmic antibodiantibodieses (ANCAs) are a staining antineutrophil group of autoantibodiesautoantibodies,, mainly of the IgGtype, against cytoplasmic antibodies, antigens in the cytoplasmcytoplasm of neutrophilsand monocytes.monocytes. show a perinuclear They are detected in a number of conditions associated staining pattern. with systemic vasculitisvasculitis,, so called ANCEANCE--associatedassociated c-ANCA, or classical vasculiditiesvasculidities,, including Wegener’s granulomatosis; antineutrophil cytoplasmic microscopic polyangitis and Churg–Strauss syndrome. antibodies, show a ANCA were originally shown to divide into two main diffusely granular, classes, cc--ANCAANCA and pp--ANCA,ANCA, based on the pattern of cytoplasmic staining staining on ethanol-ethanol-fixedfixed neutrophils and the main target pattern. antigen. ANCA titers can also be measured using ELISA It is cc--ANCAANCA that is primarily and indirect immunofluorescence.immunofluorescence. associated with Wegener’s
Posterior Scleritis Summary of Work-up
Vascular congestion with hemorrhage, reduced Patients with scleritis need to be evaluated for potential systemic vision with macular stria – but with PAIN! diseases. I would recommend that the scleritis work-up be done by a rheumatologist due to the wide range of conditions. Laboratory studies are likely to include both serologic tests and urinalysis. Luetic serology – FTA-Abs and VDRL/RPR CBC with diff Anti-neutrophil cytoplasmic antibody (ANCA) studies will test for Wegener’s granulomatosis. Various vasculidities can affect kidney and so urine protein should be measured. R/o TB with CXR and PPD
B-scan: Flattening of posterior pole, Choroidal and scleral thickening with sub-Tenon’s fluid accumulation and “T-sign”
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Based on the past history, review of systems, and physical examination, select appropriate diagnostic tests to confirm or reject the following suspected associated diseases: Treatment of Scleritis
Rheumatoid factor - Rheumatoid arthritis Oral NSAIDS first line, either COX-1 or more Antinuclear antibodies - Systemic lupus erythematosus,erythematosus, rheumatoid arthritis, polymyositispolymyositis,, progressive systemic sclerosis, or mixed connective tissue recent COX-2 such as Celebrex at 100-200 mg Antineutrophil cytoplasmic antibodies (ANCA) - Wegener granulomatosisgranulomatosis,, polyarteritis nodosanodosa,, or microscopic polyangiitis qd. More difficult cases may require oral Human leukocyte antigen (HLA) typing - Ankylosing spondylitis, reactive arthritis , steroids or immunosuppressants. If NSAID use psoriatic arthritis, or arthritis associated with inflammatory bowel disease Eosinophil count/immunoglobulin E ((IgEIgE)) - Allergic angiitis of Churg-Churg-StraussStrauss chronic, protection of GI system may be syndrome or atopy required (omeprazole-Prilosec) Uric acid -Gout Erythrocyte sedimentation rate (ESR) - Giant cell arteritis Treatment should be coordinated with Hepatitis B surface antigen ((HBsAgHBsAg)) -Polyarteritis- Polyarteritis nodosa Serologies - Infectious diseases, including syphilis and Lyme disease rheumatologist of an ophtho with specialty PurifiedPurified--proteinprotein derivative (PPD) skin test - Tuberculosis training in ocular immune disease. Anergy skin test -Sarcoidosis Prick test -Atopy- Atopy
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