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. 2021 Mar;19(3):400-426.
doi: 10.1111/ddg.14332. Epub 2021 Feb 23.

Autoinflammatory syndromes

Barbara Meier-Schiesser  1 , Lars E French  2
Affiliations

Autoinflammatory syndromes

Barbara Meier-Schiesser et al. J Dtsch Dermatol Ges. 2021 Mar.

Abstract

Autoinflammatory syndromes are a steadily growing group of inflammatory diseases caused by abnormal regulations of the innate immune system. The clinical presentation is multifaceted, but recurrent fever, skin involvement, joint inflammation and other systemic symptoms of inflammation are characteristic. In contrast to classic autoimmune diseases, autoantibodies or specific T cells are not involved in the pathogenesis. In fact, innate immunity plays the most important role in autoinflammation. While activation of the innate immune system is usually self-limiting in healthy individuals, mutations and dysregulation can lead to chronic and excessive activation of innate immune responses and to the development of autoinflammatory diseases.

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