Catalog

Schnitzler syndrome

No MMS code

Link to WHO Definition

Schnitzler syndrome – http://id.who.int/icd/entity/1867840545

Schnitzler syndrome is a rare acquired auto-inflammatory disorder characterised by recurrent fever, urticaria, fatigue, bone and joint pain, lymphadenopathy, leukocytosis and a monoclonal IgM component. Mean age of onset is around 50. The disease follows a chronic course. A large number of immunosuppressive agents are ineffective but the IL-1 receptor antagonist anakinra appears to provide rapid and sustained relief. The monoclonal IgM component is a defining feature and may result in AA amyloidosis. There is a risk of eventual lymphoproliferative complications such as lymphoma, IgM myeloma or Waldenström macroglobulinaemia.