Case series: rituximab in the treatment of refractory sight-threatening scleritis

Purpose

Scleritis is a chronic vasculitis of scleral vessels leading to a substantial amount of morbidity and even blindness. Oral steroids and intensive immunosuppressive treatments are often used to to achieve long-term control of disease. But those drugs have severe adverse effects and there is a significant number of non-responders. We describe six patients with refractory scleritis who received rituximab.

Methods

A case series of six patients (aged 39-66) with refractory scleritis (4 idiopathic, 2 relapsing polychondritis), including B cell monitoring. One treatment cycle consisted of 1000 mg initially and 1000 mg two week later. Prior to infusion 100 mg methyprednisolon was administered i.v.

Results

In all patients disease activity decreased. However, in four patients symptoms returned resp. 2,4,6 and 9 months after therapy. In two cases complete remission was achieved. Two patients received a second rituximab cycle successfully. No correlation was found between B cells reoccurrence and refractory disease. [Figure 1]

Figure 1

Conclusions

Clinical activity of scleritis in all patients decreased after treatment. In two cases scleritis went in complete remission after one cycle of treatment. Rituximab may be a new promising therapeutic tool in the treatment of refractory scleritis. There is no correlation between relapses and B cell recovery.