{"title":"施尼茨勒综合征","authors":"F F Gellrich, C Günther","doi":"10.1007/s00105-019-4434-4","DOIUrl":null,"url":null,"abstract":"<p><p>Schnitzler syndrome is a very rare acquired systemic disease with many similarities to hereditary autoinflammatory syndromes. The main characteristics are generalized exanthema and IgM monoclonal gammopathy. Other clinical features include fever, muscle, bone, and/or joint pain, and lymphadenopathy. About 15-20% of patients with Schnitzler syndrome develop lymphoproliferative diseases and, in rare cases, amyloid A (AA) amyloidosis can occur if the disease is not treated. Activation of the innate immune system, especially interleukin (IL)-1β, is central to the pathogenesis of disease. Consequently, complete control of disease symptoms can be achieved in 80% of patients by treatment with the IL-1 receptor antagonist anakinra.</p>","PeriodicalId":12970,"journal":{"name":"Hautarzt","volume":" ","pages":"6-11"},"PeriodicalIF":0.0000,"publicationDate":"2025-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":"{\"title\":\"Schnitzler syndrome.\",\"authors\":\"F F Gellrich, C Günther\",\"doi\":\"10.1007/s00105-019-4434-4\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"<p><p>Schnitzler syndrome is a very rare acquired systemic disease with many similarities to hereditary autoinflammatory syndromes. The main characteristics are generalized exanthema and IgM monoclonal gammopathy. Other clinical features include fever, muscle, bone, and/or joint pain, and lymphadenopathy. About 15-20% of patients with Schnitzler syndrome develop lymphoproliferative diseases and, in rare cases, amyloid A (AA) amyloidosis can occur if the disease is not treated. Activation of the innate immune system, especially interleukin (IL)-1β, is central to the pathogenesis of disease. Consequently, complete control of disease symptoms can be achieved in 80% of patients by treatment with the IL-1 receptor antagonist anakinra.</p>\",\"PeriodicalId\":12970,\"journal\":{\"name\":\"Hautarzt\",\"volume\":\" \",\"pages\":\"6-11\"},\"PeriodicalIF\":0.0000,\"publicationDate\":\"2025-06-01\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"Hautarzt\",\"FirstCategoryId\":\"3\",\"ListUrlMain\":\"https://doi.org/10.1007/s00105-019-4434-4\",\"RegionNum\":4,\"RegionCategory\":\"医学\",\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"\",\"PubModel\":\"\",\"JCR\":\"Q3\",\"JCRName\":\"Medicine\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"Hautarzt","FirstCategoryId":"3","ListUrlMain":"https://doi.org/10.1007/s00105-019-4434-4","RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q3","JCRName":"Medicine","Score":null,"Total":0}
Schnitzler syndrome is a very rare acquired systemic disease with many similarities to hereditary autoinflammatory syndromes. The main characteristics are generalized exanthema and IgM monoclonal gammopathy. Other clinical features include fever, muscle, bone, and/or joint pain, and lymphadenopathy. About 15-20% of patients with Schnitzler syndrome develop lymphoproliferative diseases and, in rare cases, amyloid A (AA) amyloidosis can occur if the disease is not treated. Activation of the innate immune system, especially interleukin (IL)-1β, is central to the pathogenesis of disease. Consequently, complete control of disease symptoms can be achieved in 80% of patients by treatment with the IL-1 receptor antagonist anakinra.
期刊介绍:
Der Hautarzt is an internationally recognized journal informing all dermatologists working in practical or clinical environments about important developments in the field of dermatology including allergology, venereology and related areas.
Comprehensive reviews on a specific topical issue focus on providing evidenced based information on diagnostics and therapy.
Freely submitted original papers allow the presentation of important clinical studies and serve the scientific exchange.
Case reports feature interesting cases and aim at optimizing diagnostic and therapeutic strategies.
Review articles under the rubric "Continuing Medical Education" present verified results of scientific research and their integration into daily practice.