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- Title
Autoinflammation – Unterschiede bei Kindern und Erwachsenen.
- Authors
Krusche, Martin; Kallinich, Tilmann
- Abstract
Autoinflammatory diseases present as multisystemic inflammation and often manifest in early childhood. In contrast, in a few diseases, e.g., the recently described VEXAS (vacuoles, E1 enzyme, X‑linked, autoinflammatory, somatic) syndrome, the first symptoms occur exclusively in adulthood. This article describes how the phenotypic expression and severity of individual autoinflammatory diseases differ depending on age. Furthermore, differences in the development of organ damage in children and adults are pointed out. In addition to the hereditary periodic fever syndromes, the clinical picture of deficiency of adenosine deaminase 2, the interferonopathies, periodic fever, aphthous stomatitis, pharyngitis, and adenitis syndrome as well as VEXAS and Schnitzler syndromes are highlighted.
- Subjects
ADENOSINE deaminase; PHARYNGITIS; AUTOINFLAMMATORY diseases; MORPHOGENESIS; MEVALONATE kinase; FAMILIAL Mediterranean fever
- Publication
Zeitschrift für Rheumatologie, 2022, Vol 81, Issue 1, p45
- ISSN
0340-1855
- Publication type
Article
- DOI
10.1007/s00393-021-01115-y