Conflicts of interest: none declared. Sir, The Schnitzler syndrome is a rare condition characterized by chronic urticaria in association with monoclonal IgM gammopathy. Other features of the syndrome are fever, elevated erythrocyte sedimentation rate (ESR), arthralgia, bone pain, osteosclerotic bone lesions, leucocytosis, hepatosplenomegaly and lymphadenopathy. Therapy is often unsatisfactory,1, 2 and to date no spontaneous complete remissions have been reported. We describe a patient who developed AA amyloidosis 18 years after presentation. In 1986, a 54‐year‐old man presented to the rheumatology department with a 3‐year history of bone pain accompanied by fever. Laboratory examination revealed an elevated ESR (69 mm in the first hour) and a slightly elevated level of IgM (4 g L−1) (normal 0·57–3·4). Bone marrow examination showed a normal plasmacytosis of 4% (normal 0–5%). Bone scintigraphy showed pathological bone turnover in both knees and left femur with bilateral fibrostosis on spot X‐rays. A tentative diagnosis of osteomyelitis was made. Because ESR and leucocyte count remained elevated, despite antibiotic therapy treatment with nonsteroidal anti‐inflammatory drugs (NSAIDs) was initiated. This gave a marked improvement of his symptoms but his inflammatory parameters remained high. He also developed nonpruritic urticarial lesions that did not respond to the treatment with NSAIDs.
No takes yet. Share an insight, caveat, or question.
Claes et al. (2007) studied this question.