Dramatic response to IL1-RA treatment in longstanding multidrug resistant Schnitzler's syndrome: a case report and literature review.

Clinical Rheumatology
Emilio Besada, Hans Nossent

Abstract

We report a case of longstanding multidrug resistant Schnitzler's syndrome that finally went into clinical remission upon treatment with anakinra and review the literature concerning IL1-RA treatment for Schnitzler's syndrome. A now 71-year-old patient presenting with recurring episodes of urticaria and fever and secondary weight loss for the past 16 years as well as arthralgia, hearing loss. The patient has anemia, leucocytosis with neutrophilia, thrombocytosis, elevated C-reactive protein and erythrocyte sedimentation rate, lymphadenopathy and a monoclonal IgM kappa band that later became oligoclonal with two IgM kappa bands and one IgM lambda band. The patient was treated with moderate effect with combination of prednisolone, azathioprine, and colchicine. In March 2009, anakinra 100 mg daily sc was added during a disease flare. Within 24 h after the first injection, both the urticaria and the fever disappeared and have not recurred. For the past 6 months, the patient has been in clinical and biochemical remission. Colchicine has been stopped while the azathioprine and prednisolone doses are being reduced. Twenty-four patients with Schnitzler's syndrome, including three patients with a variant of Schnitzler's syndrome and thr...Continue Reading

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