|Year : 2015 | Volume
| Issue : 1 | Page : 54-55
Nodular scleritis as the eye manifestation in Behcet's syndrome
Karpagam Damodaran1, Parthopratim Dutta Majumder1, Jyotirmay Biswas2
1 Department of Uvea and Intraocular Inflammations, Sankara Nethralaya, Chennai, India
2 Director of Uveitis and Ocular Pathology Departments,Sankara Nethralaya, Chennai, India
|Date of Web Publication||23-Jan-2015|
Senior resident ,Department of Uvea and Intraocular Inflammations Sankara Nethralaya No. 41, College Road, Nungambakkam, Chennai
Source of Support: None, Conflict of Interest: None
| Abstract|| |
We report a case of a 45-year-old female, who was diagnosed as Behcet's disease and presented to us with nodular scleritis in her right eye.
Keywords: Behcets, human leukocyte antigen B51, nodular scleritis
|How to cite this article:|
Damodaran K, Majumder PD, Biswas J. Nodular scleritis as the eye manifestation in Behcet's syndrome. Oman J Ophthalmol 2015;8:54-5
| Introduction|| |
Scleritis is known to be associated with systemic connective tissue diseases or vasculitic diseases such as rheumatoid arthritis (RA), Wegener's granulomatosis, relapsing polychondritis, and systemic lupus,other less frequently associated diseases include polyarteritis nodosa, reactive arthritis, psoriatic arthritis, giant cell arteritis and Cogan syndrome.The scleritis occurred in patients with Behcets disease has been rarely reported in the literature. We describe a female patient who developed the scleritis in the course of Behcets disease.
| Case Report|| |
A 45-year-old female presented to us with chief complaints of pain and redness in her right eye for the past 1 month. She was diagnosed as having Behcet's syndrome by a rheumatologist elsewhere 4 years ago when she had been suffering from recurrent oral and genital ulcers. During the course of her disease she also developed acne like pustular skin eruptions, erythema nodosum like lesions and arthritis predominately involving her knees and a positive pathergy test. She also gave a history of intermittent painful redness alternating in both her eyes, initially in her left eye and later in the right eye. She was being treated with colchicine (1.5 mg/day), deflazacort and dapsone systemically for the past 4 years. However, when she presented to us she was totally off all medications.
Ophthalmic examination revealed a best-corrected visual acuity of 20/20 in both her eyes. Intraocular pressures by Goldman applanation tonometry were 12 and 14 mm of Hg in her right and left eye respectively. Slit lamp examination of the right eye revealed a tender scleral nodule temporally along with marked dilatation of the deeper episcleral vessels [Figure 1]. The rest of the anterior segment evaluation was normal in both the eyes. Fundus examinations of both her eyes were normal. Fundus fluorescein angiography did not reveal any vascular leakage or abnormality [Figure 2].
|Figure 1: Slit lamp examination of the right eye showing scleral nodule with deep episcleral congestion|
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Physical examination was however not contributory at the time of her presentation. Laboratory investigations showed raised erythrocyte sedimentation rate, anaemia and leukocytosis. She was found to be negative for human leukocyte antigen-B51 and 5. All other investigations, including rheumatoid factor, antinuclear antibody, anti-neutrophil cytoplasmic antibodies (c and P anti-neutrophil cytoplasmic antibodies) were negative. She was diagnosed nodular scleritis in her right eye and after consultation with a rheumatologist, she was started on oral prednisolone 30 mg/day, to be tapered gradually along with oral azathioprine 50 mg twice a day.
| Discussion|| |
Behcet's disease is a chronic, relapsing, multisystem, idiopathic inflammatory disorder characterized by a triad of oral ulcers, genital ulcers, and ocular lesions. However, the spectrum of disease is even wider with the involvement of articular, intestinal, vascular, urogenital, and neurologic systems, besides the mucocutaneous and ocular findings. , The disease has a higher prevalence particularly in Turkey, Iraq, Iran, Korea, and Japan and accounts for up to 20% of cases of endogenous uveitis in some of these countries. , The diagnosis of Behcet's disease is based on the identification of its typical clinical features, according to the diagnostic criteria laid down by the International Study Group and the Japanese criteria for the diagnosis of Behcet's disease. ,
Previously published reports on Behcet's disease among Indian patients highlight the fact that Behcet's disease, as well as ocular involvement, is significantly less common in Indian population. ,,, In a study of 53 patients at a tertiary care centre in India by Sachdev et al.,  eight patients (16.3%) had isolated anterior uveitis, while 13 patients (26.5%) had predominantly posterior uveitis and the remaining 28 patients (57.1%) had panuveitis. Those with anterior segment involvement predominantly consisted of hypopyon uveitis and two cases of conjunctival ulceration. This was in contrast to a study by Rohatgi and Singal  who reported that the most common ocular manifestation in their patients was conjunctival ulceration (26.3%), while uveitis was a rare finding (10.5%).
Scleritis is associated with systemic connective tissue diseases and/or vasculitic problems, such as rheumatoid arthritis, Wegener's granulomatosis, polyarteritis nodosa, relapsing polychondritis and systemic lupus erythematosus in approximately 50% of cases.  To our knowledge only four cases of scleritis associated with Behçet's disease have been reported in the literature in the past.
Chang and Cho reported a 46-year-old woman presenting with nodular scleritis  and Sainz de la Maza et al. reported one out of 172 patients.  Sakellariou et al. reported a case of scleromalacia perforans in a patient with Behcets disease.  Akova et al. have also reported an atypical case of myositis with scleritis in association with Behcet's disease. 
Cases of diffuse or nodular scleritis that show resistance to conventional therapy or recur very frequently should be investigated carefully. The association of scleritis in Behçet's disease may suggest the aggressive use of immunosuppressive agents earlier in the disease process, two or three immunsupressive agents may be considered. Aggressive systemic therapy is necessary to save not only the eye but also the patient's life.
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[Figure 1], [Figure 2]