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Schnitzler syndrome

Etiology: - acquired disorder associated with MGUS Epidemiology: - 1% among patients with MGUS Pathology: - rash with neutrophilic infiltrate Clinical manifestations: - IgM monoclonal gammopathy - chronic urticaria - fever (75%) - fatigue (75%) - arthralgia (68%), bone pain (63%) - daily attacks of arthritis & bone pain - night sweats (25%) - lymphadenophathy (44%) - hepatosplenomegaly - peripheral neuropathy (54%) * a long delay between the onset of symptoms & diagnosis, often up to 5 years, is common Laboratory: - no diagnostic tests* - complete blood count (CBC) - leukocytosis - serum protein electrophoresis - IgM-kappa monoclonal gammopathy (94%) Complications: - development of lymphoproliferative disease is rare - may evolve into Waldenstrom's macroglobulinemia Management: - interleukin 1-beta inhibitor [1] - rilonacept - canakinumab (Ilaris) - anakira

General

autoimmune disease syndrome

References

  1. Jain T et al. Schnitzler syndrome: An under-diagnosed clinical entity. Haematologica 2013 Oct; 98:1581 PMID: 23812931 http://www.haematologica.org/content/98/10/1581
  2. Medical Knowledge Self Assessment Program (MKSAP) 18, American College of Physicians, Philadelphia 2018