Abstract
Purpose: :
Scleritis is a rare chronic ocular vasculitis of scleral vessels leading to a substantial amount of morbidity and even blindness. Oral steroids are widely used to treat scleritis and some patients will require intensive immunosuppressive treatment to achieve long-term control of disease. The use of those drugs is limited by adverse effects such as life-threatening infections and malignancies. Moreover, a significant number of patients does not respond. We describe two patients with refractory anterior scleritis responding to B cell ablative therapy (rituximab).
Methods: :
A case series of two patients with therapy refractory anterior scleritis.
Results: :
Patient 1: A 36-year old female with relapsing polychondritis developed scleritis of the right eye, with photophobia and deteriorating vision. During a period of 18 months she received several immunosuppressives including oral steroids, methotrexate, mycophenolate sodium (MPS), high dose solumedrol, infliximab, cyclophosphamide, and intravenous immunoglobulins (IVIG). Although the other symptoms responded, the scleritis persisted. This patient received two doses of 1000 mg rituximab with a two-week interval whilst continuing MPS and IVIG. Two months hereafter the scleritis improved and pain resolved. This was accompanied by an significant reduction of peripheral B lymphocyte for at least 6 months. Patient 2: A 39-year old man was diagnosed with bilateral idiopathic scleritis. Treatment in course of years included high dose steroids, diafenylsulfon, azathioprine, methotrexate, cyclophosphamide and adalimumab. The scleritis only briefly responded and the patient became dependent on high doses of steroids, with unacceptable adverse effects. Ten years after onset of disease, treatment with two doses of 1000 mg rituximab and subcutaneous immunoglobulins was initiated. Within 3 months the inflammation resolved completely and steroids could be tapered. Peripheral B lymphocytes were undetectable for at least 10 months and recovered after 17 months. To date (2 years after rituximab therapy) no relapse occurred.
Conclusions: :
Clinical activity of scleritis in both patients with severe therapy refractory scleritis disappeared after B cell ablative therapy. Rituximab may be a new promising therapeutic tool in the treatment of refractory scleritis.
Keywords: autoimmune disease