Preferred Label : Schnitzler syndrome;
ICD-11 definition : Schnitzler syndrome is a rare acquired auto-inflammatory disorder characterized 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.;
ICD-11 synonym : Chronic urticaria with macroglobulinaemia;
Origin ID : 1867840545;
UMLS CUI : C0524988;
Currated CISMeF NLP mapping
- Semantic type(s)
- UMLS correspondences (same concept)
Schnitzler syndrome is a rare acquired auto-inflammatory disorder characterized 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.
