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Abstract
A 40-year-old woman had been suffering of many symptoms of Behcet’s disease (BD) since her childhood without being diagnosed. She had recurrent mouth ulcers and from the age of 21 vulva ulcers followed by loss of vision due to central retinitis and later also ankle arthritis. At the age of 31, uveitis due to BD was diagnosed. The fact that BD is often not recognized in Bangladesh explains the marked delay in her diagnosis. The initial central retinitis responded well to oral glucocorticoids, azathioprine and cyclosporine, but in the course of time she became refractory to these agents. Considering her financial constraints, low dose Infliximab (3mg/kg) was used resulting in a remarkable but temporal improvement, she came in remission by using 5mg/kg regimen with increased interval time. We summarize the treatment options for ocular involvement in BD patients Conclusions: In countries like Bangladesh diagnosis of Behcet’s disease can be delayed. Early cooperation of dermatologists and eye surgeons with rheumatologists is necessary. Most cases of resistant ocular BD can nowadays be successfully treated with anti TNF-agents. Promising results are published with other biologics for refractory and multi-resistant cases.
Highlights
Behçet’s disease (Morbus Behçet) is known as Behçet’s syndrome, Behçet-Adamantides syndrome, or Silk Road disease [1]
A 40-year-old woman had been suffering from many symptoms of Behçet’s disease (BD) since her childhood without being diagnosed. She had recurrent mouth ulcers and, from the age of 21, vulva ulcers followed by loss of vision due to central retinitis and later ankle arthritis
BD combined with uveitis is rare in Southeast Asia; in a worldwide survey 54% of cases were from the Middle East, 43% were from Europe, and only 2% were from East- and South Asian countries [13]
Summary
Behçet’s disease (Morbus Behçet) is known as Behçet’s syndrome, Behçet-Adamantides syndrome, or Silk Road disease [1]. In July 2002, the patient noticed some visual impairment, and from January 2003, she saw tiny black shadows while reading and only a half view of the wall clock in her right eye Her ophthalmologist diagnosed central retinitis and prescribed prednisolone 40 mg/day, tapered by 5 mg every week to 15 mg/day as a maintenance dose over a period of 1 month. She was put on oral prednisolone and azathioprine (AZA) with corticosteroid eye drops and mydriatics She improved, but when the prednisolone was tapered to below 15 mg/day, the disease flared aggressively despite continuing of AZA 2 mg/ kg. But when the prednisolone was tapered to below 15 mg/day, the disease flared aggressively despite continuing of AZA 2 mg/ kg To treat her ocular flare and for corticosteroid sparing, cyclosporine was added. 17.04.16 Quiet Quiet + 15.10.16 Quiet Quiet + 13.12.16 Quiet Quiet + 21.7.18 Quiet Quiet +
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