Multiorgan failure and macrophage activation syndrome in adult-onset Still´s disease


Authors: J. Tomš 1;  T. Soukup 1;  J. Polák 2;  P. Bradna 1
Authors‘ workplace: 2. interní klinika, oddělení revmatologie, Lékařská fakulta UK a Fakultní nemocnice Hradec Králové 1;  Klinika anesteziologie, resuscitace a intenzivní medicíny, Lékařská fakulta UK a Fakultní nemocnice Hradec Králové 2
Published in: Čes. Revmatol., 20, 2012, No. 3, p. 133-137.
Category: Case Report

Overview

Macrophage activation syndrome (MAS) is a relatively rare and potentially life threatening complication of autoimmune systemic diseases, mainly systemic juvenile idiopathic arthritis. It is characterized by persistent fever, cytopenia, internal organ dysfunction and coagulopathy on the basis of activation and proliferation of T-lymphocytes and macrophages with uncontrolled cytokine release (so called „cytokine storm“). The authors present a case of 36-year-old woman with simultaneous manifestation of adult-onset Stillęs disease and MAS. Multiorgan failure required a complex intensive care, lasting several weeks. The combined immunosuppressive therapy led to the remission of both diseases.

Key words:
macrophage activation syndrome, adult-onset Stillęs disease, multiorgan failure


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Labels
Dermatology & STDs Paediatric rheumatology Rheumatology
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