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To the Editor: Interleukin 1 (IL-1) has been identified to mediate several autoinflammatory diseases with features of chronic urticaria, polyarthritis, and systemic inflammation1. A case clinically resembling Schnitzler’s syndrome but with polyclonal IgA is described. The rapid response to IL-1 blockade with clinical remission indicates a critical role of IL-1 in the pathogenesis of this disorder.A 54-year-old white woman presented with chronic urticaria and joint pain. Urticaria began at 9 years of age and bilateral knee joint pain began 3 years later. These symptoms had been recurrent and appeared more frequently with age, from once a week to once every 2–3 days, and lasted longer, from one day to 2–3 days and at times sustained for one week. One year before presentation, mildly pruritic urticaria appeared daily, first in the trunk, and spread to the extremities. Her joint pain was associated with rash and involved knees, ankles, shoulders, elbows, and occasionally wrists and proximal interphalangeal joints. Inflammatory synovial fluid without crystals was detected in the left knee 5 months prior to … Address correspondence to Dr. C-Q. Chu, Division of Arthritis and Rheumatic Diseases, Oregon Health and Science University, 3181 SW Sam Jackson Park Road, OP09, Portland, OR 97239, USA. E-mail: chuc{at}ohsu.edu

Chronic Urticaria and Arthritis with Polyclonal IgA: Rapid Response and Clinical Remission with Interleukin 1 Blockade: Figure 1.