[Still's disease in children and adults].

Abstract:

:Systemic juvenile idiopathic arthritis (sJIA) is characterized by fever, arthritis, and other signs of systemic inflammation. Historically, sJIA was named Still's disease after George Frederic Still, who first reported patients. Individuals who manifest after the 16th birthday are diagnosed with adult onset Still's disease (AOSD). The pathophysiology of sJIA and AOSD are incompletely understood. Increased activation of inflammasomes and the expression of proinflammatory cytokines play a central role. S100 proteins, which can activate Toll-like receptors, thus, maintaining positive feedback loops, have also been detected at increased levels in sera from sJIA patients. Reduced expression of the immune-modulatory cytokine IL-10 may further contribute to immune cell activation and the production of proinflammatory molecules. Here, we discuss the clinical picture, differential diagnoses, the current pathophysiological understanding, and treatment options in sJIA and AOSD.

journal_name

Z Rheumatol

authors

Hedrich CM,Günther C,Aringer M

doi

10.1007/s00393-017-0358-5

subject

Has Abstract

pub_date

2017-09-01 00:00:00

pages

595-608

issue

7

eissn

0340-1855

issn

1435-1250

pii

10.1007/s00393-017-0358-5

journal_volume

76

pub_type

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