gms | German Medical Science

In primary Sjögrens-syndrome (PSS) T cell mediated autoimmunity plays a significant role in immunopathogenesis. However, B cell mediated immunity in addition has been shown important. Therefore, a B cell directed immunosuppression seems reasonable in treatment resistant cases.

A 60-year-old woman suffered from PSS with keratoconjunctivitis sicca, chronic anterior scleritis and arthralgia. She needed systemic corticosteroids (> 30mg/d) and various other immunosuppressants (methotrexate 20mg/week i.v., cyclosporine A 5mg/kg/d p.o., cyclophosphamide 2mg/kg/d p.o., intravenous immunoglobulins 0.4g/kg, TNF-α inhibitor infliximab 5mg/kg i.v., leflunomide 20mg/d p.o.). These substances were applied in various combinations within 2 years without induction of remission of scleritis. After a 4 week interruption of that treatment, still under 50mg/d prednisolone, infusion with Mabthera^© (Rituximab, anti-CD20 chimaeric, monoclonal antibody, 375mg/m²) was given. The patient got four cycles of rituximab infusions at a four week interval. The scleritis disappeared with improvement of sicca-symptoms and arthralgia. Corticosteroids could be reduced to 5mg/d. FACS-analysis of peripheral lymphocytes showed a depletion of CD-20 positive B cells. Meanwhile maintenance of remission is performed with mycophenolic acid/mofetil (2g/d).

B lymphocyte cell depletion with rituximab seems a new treatment option in refractory scleritis due to Sjögrens syndrome. It seems reasonable to test this treatment strategy in more patients.