Autoinflammation in der Dermatologie
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- 作者：Dr. Peter Maximilian Heil
- 关键词：SchlüsselwörterAutoinflammation ; Inflammasom ; Angeborenes Immunsystem ; Serum Amyloid A ; Fieber
- 刊名：hautnah
- 出版年：2017
- 出版时间：February 2017
- 年：2017
- 卷：16
- 期：1
- 页码：24-30
- 全文大小：377KB
- 刊物主题：Dermatology; Medicine/Public Health, general;
- 出版者：Springer Vienna
- ISSN：2192-6484
- 卷排序：16
文摘
Autoinflammatory syndromes are rare – often monogenic – overreactions of the inborn immune system, in particular of monocytes and macrophages (this is in contrast to autoimmunity as a deviation of the adaptive immune system, represented by antigen-specific T cells and B cells). In the pathogenic center of most autoinflammatory diseases is the so-called inflammasome; this is a cellular assembly of proteins dedicated to sensing danger signals. In the case of autoinflammation, mutations of inflammasome proteins result in spontaneous activation and subsequent overproduction of IL1‑β. Onset is during childhood; it sometimes occurs in neonates, but rarely in adults. Common symptoms are episodes of fever, arthralgias, and urticarial exanthemas; the latter can be clearly differentiated from classic urticaria (e. g., pain instead of itch). Apart from these common symptoms, there are signs specific for certain autoinflammatory syndromes (e. g., pseudocellulitis in familial Mediterranean fever). Such constellations should raise suspicion of an autoinflammatory syndrome. Helpful laboratory values are elevated sedimentation rate and CRP, neutrophilic leukocytosis and elevated serum amyloid A. Definite diagnoses are based on genetic investigations or specific signs and symptoms. In most cases, the therapy of choice is blocking interleukin 1; several s. c. compounds are available for this purpose, e. g., anakinra, canakinumab, and rilonacept. The most important long-term sequela of an untreated autoinflammatory syndrome is amyloidosis (heart, kidney).