Abstract
Purpose: :
To evaluate clinical response to Rituximab (RTX) in 4 refractory patients with necrotizing scleritis secondary to Wegener granulomatosis.
Methods: :
We studied 4 patients with Wegener granulomatosis acording to the Chapel Hill criteria and severe necrotizing scleritis unresponsive to steroids and immunesupressors treated with RTX one gram twice, two-weeks apart. We evaluated clinical response, lymphocyte subsets and anti-neutrophil citoplasm antibody.
Results: :
Case1: A 51 years old male with refractory necrotizing scleritis who received 103gr cumulative cyclophosphamide (CYC). Azathioprine (AZA) was not tolerated and prednisone (PDN) remained >30mg qd.Case 2: A 36 years old female with rhinosinusitis and necrotizing scleritis with peripheral ulcerative keratitis. She had received high-dose PDN, CYC (120gr), methotrexate (MTX) and AZA. In spite of PDN 60mg qd she had active sinusitis and corneal perforation.Case 3: A 56 years old female with severe necrotizing scleritis. After years, PDN remained >30mg qd, had severe Cushing and did not respond to MTX or CYC. A refractory panuveitis developed.Case 4: A 28 years old male with progressive and severe peripheral ulcerative keratitis with perforation associated to necrotizing scleritis, rhinosinusitis and pulmonary afection despite high-dose PDN and CYC.Evolution: RTX was well tolerated in all cases, There was an immediate lymphocyte depletion without concomitant infections. Clinical improvement was seen in the first 4 weeks without progresion of ocular melting process. At 8 months all patients were of other immunesupressors.
Conclusions: :
MTX is useful for the treatment for refractory necrotizing scleritis in patients with Wegener granulomatosis
Keywords: sclera • clinical (human) or epidemiologic studies: treatment/prevention assessment/controlled clinical trials • keratitis