Case Communications Episcleritis Associated with Familial Mediterranean Fever Svetlana Berestizschevsky MD, Dov Weinberger MD, Inbal Avisar MD and Rahamim Avisar MD External Eye Disease Clinic, Department of Ophthalmology, Rabin Medical Center (Golda Campus), Petah Tikva, and Sackler Faculty of Medicine, Tel Aviv University, Ramat Aviv, Israel Key words: episcleritis, familial Mediterranean fever, pseudopterygium, symblepharon IMAJ 2008;10:318–319 Familial Mediterranean fever is an auto- somal recessive disease found predomi- nantly in individuals of Jewish Sephardic∗, Armenian, Turkish and Arabic ancestry. It is characterized by recurrent episodes of fever, peritonitis and/or pleuritis. Arthritis, skin lesions and amyloidosis are seen in some patients, the latter leading to nephrotic syndrome and uremia. Attacks of FMF resolve spontaneously, but A B treatment with colchicine is required to [A] Nodular episcleritis. [B] B-scan echo of the left eye. prevent the development of amyloidosis. Reports of ophthalmological manifesta- tions in FMF are few and include retinal 0.0–0.5). She had normochromic and systemic corticosteroids and oral non- colloid-like bodies, panuveitis, anterior normocytic anemia with hemoglobin of steroidal anti-inflammatory drugs for uveitis, scleritis, episcleritis and papillitis. 11.0 g/dl. Assays for antinuclear antibody, one month. The oral colchicine dose was We describe a patient with FMF who pre- anti-dsDNA, rheumatoid factor, human im- increased to 2 mg daily. At follow-up, sented with nodular episcleritis that was munodeficiency virus and hepatitis antigen symblepharon and pseudopterygium at the successfully treated with local steroids were negative. Routine serum chemistry 6 o’clock position were detected. Injection and oral colchicine. The English-language tests and urinalysis were normal. Serum of 0.1 ml of 0.15 mg/ml mitomycin C into medical literature on episcleritis in FMF protein electrophoreses was normal. The the symblepharon tissue failed to induce is reviewed. fibrinogen level at that time was 321 mg/dl any improvement. Therefore, 6 months (200–400 mg/dl). Tissue typing of the pa- later, the pseudopterygium and cicatricle Patient Description tient for human leukocyte antigen-B27 was tissue of the symblepharon were widely A 40 year old Arab woman with FMF negative. We did not perform molecular excised, and amniotic membrane was presented with redness and irritation analysis of her DNA for possible muta- transplanted over the bar sclera. At the 4 of the left eye of 6 days duration. She tions in the MEFV gene. Radiographs of months postoperative follow-up, there was was being treated with prophylactic the hands and elbows were normal. Her no recurrence of the pseudopterygium, colchicine (tablets 0.5 mg x 3 per day) pelvis X-ray showed no sign of sacro- and deep lower fornix was formed. since age 12. She had no abdominal iliitis. Best corrected visual acuity was 6/6 symptomatology related to inflammatory in both eyes and intraocular pressure was Comment gastrointestinal disease. Examinations by normal. Slit-lamp examination revealed no FMF is very often associated with other physicians from the departments of abnormalities in the anterior or posterior autoimmune diseases such as ankylos- pulmonary diseases and cardiology were segments of the right eye. The left eye ing spondylitis, rheumatoid arthritis and also unremarkable. The remainder of her showed necrotizing blepharitis combined others, which are chronic progressive dis- physical examination was unremarkable. with nodular episcleritis [Figure A]. Tissue eases of unknown etiology [1]. FMF may Laboratory studies showed that erythro- biopsy of the episcleral lesion revealed a manifest as arthritis, pleuritis, pericardits, cyte sedimentation rate was 70 mm/hour non-specific inflammation with moderate orchitis or peritonitis. The association and C-reactive protein 0.6 mg/dl (normal dysplasia. No amyloidosis was observed. of familial Mediterranean fever with On B-scan echo of the left eye, thickening juvenile idiopathic arthritis or ankylosing ∗ Originating in the Middle East or North of the localized episcleral tissue was noted spondylitis, most commonly with negative Africa without scleral involvement [Figure B]. HLA-B27 antigen, has been described FMF = familial Mediterranean fever Treatment consisted of topical and in several previous reports. There is no 318 S. Berestizschevsky et al. • Vol 10 • April 2008 Case Communications specific biological marker of familial four cases of episcleritis [2-4], two cases There are many reports in the ophthalmic Mediterranean fever that is clinically of panuveitis and two cases of anterior literature that present the occurrence of available. Affected patients lack a specific uveitis have been reported in the English- episcleritis and scleritis and the relation of protease, normally present in serosal flu- language medical literature to date. Both their presence with autoimmune disease ids, that can inactivate both interleukin-8 cases of panuveitis were characterized [5]. This explains the fact that uveitis, and the chemotactic complement factor by rhegmatogenous retinal detachment. scelritis and panuveitis were reported in 5a inhibitor, but the test for this protease Topical steroids and mydriatics were FMF cases. is used only in research settings. used in most of the patients. One case Non-specific findings include increases of nodular episcleritis was treated with References in inflammatory mediators, such as serum subconjuctival tiamcenolone [3] and 1. Resnick D. Periodic, relapsing, and re- amyloid A, fibrinogen and C-reactive one case of panuveitis was treated with current disorders. In: Resnick D, ed. protein, during febrile attacks. Michaelson weekly periocular steroid injections [4]. Diagnosis of Bone and Joint Disorders. and team in 1959 were the first to report One case of flare-up of panuveitis was Philadelphia: WB Saunders Company, 1995:1150–61. eye manifestations in FMF, consisting of completely resolved following an increase 2. Yazici H, Parzarli H. Eye involvement in colloid-like bodes, in 56% of their Jewish in colchicine therapy. The present patient a patient with familial Mediterranean patients (compared to 11% of the non- showed a good response to topical and fever. J Rheumatol 1982;9:644–5. Jewish ones). Subsequent studies from the systemic corticosteroids. These findings 3. Scharf J, Meyer E, Zonic S. Episcleritis same center of a larger group of patients suggest that episcleritis and uveitis may associated with familial Mediterranean fever. Am J Ophthalmol 1985;100:337–8. yielded a lower prevalence (20%), and be associated with FMF. The report of two 4. Akman A, Varan B, Akova YA, Aydin a later review of 50 Armenian and Arab siblings [4] with FMF and eye symptoms, P. Ocular involvement in siblings with patients with FMF revealed colloid-like combined with low prevalence of eye familial Mediterranean fever. J Pediatr bodies in only 4. Consequently, ophthal- symptoms in large groups of patients may Ophthalmol Stabismus 2001;38:114–16. mic manifestations were dropped from the point to a genetic mutation specific to the 5. Watson PG, Hayreh SS. Scleritis and epis- cleritis. Br J Ophthalmol 1976;60:63–91. clinical criteria of FMF, even as a finding eye disease. to rule out this diagnosis. The patient we have described devel- In 1982, Yazici and Pazarli [2] reported oped symblepharon and pseudopterygium Correspondence: Dr. R. Avisar, External a case of anterior uveitis in a woman at 6 o’clock, despite intensive anti-inflam- Eye Disease Clinic, Dept. of Ophthalmolo- with FMF who later developed episcle- matory treatment (local and systemic). gy, Rabin Medical Center (Golda Campus), ritis. Several years later four additional This complication was challenging. P.O. Box 121, Petah Tikva 49372, Israel. cases were described, making a total Anti-fibrotic (mitomycin C) treatment for Phone: (972-3) 931-9588 of five reports of seven cases of uveitis symblepharon has been suggested. Our Fax: (972-3) 904-6848 and episcleritis in FMF patients. 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