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Open Access 01-12-2018 | Research

Diagnostic and management of life-threatening Adult-Onset Still Disease: a French nationwide multicenter study and systematic literature review

Authors: Antoine Néel, Anaïs Wahbi, Benoit Tessoulin, Julien Boileau, Dorothée Carpentier, Olivier Decaux, Laurence Fardet, Guillaume Geri, Pascal Godmer, Cécile Goujard, Hervé Maisonneuve, Arnaud Mari, Jacques Pouchot, Jean-Marc Ziza, Cédric Bretonnière, Mohamed Hamidou

Published in: Critical Care | Issue 1/2018

Abstract

Background

Adult-onset Still disease (AOSD) is a rare systemic inflammatory disorder. A few patients develop organ complications that can be life-threatening. Our objectives were to describe the disease course and phenotype of life-threatening AOSD, including response to therapy and long-term outcome.

Methods

A multicenter case series of intensive care medicine (ICU) patients with life-threatening AOSD and a systematic literature review.

Results

Twenty patients were included. ICU admission mostly occurred at disease onset (90%). Disease manifestations included fever (100%), sore throat (65%), skin rash (65%), and arthromyalgia (55%). Serum ferritin was markedly high (median: 29,110 ng/mL). Acute respiratory failure, shock and multiple organ failure occurred in 15 (75%), 10 (50%), and 7 (35%) cases, respectively. Hemophagocytosis was demonstrated in eight cases. Two patients died. Treatment delay was significant. All patients received corticosteroids. Response rate was 50%. As second-line, intravenous immunoglobulins were ineffective. Anakinra was highly effective. After ICU discharge, most patients required additional treatment. Literature analysis included 79 cases of AOSD with organ manifestations, which mainly included reactive hemophagocytic syndrome (42%), acute respiratory failure (34%), and cardiac complications (23%). Response rate to corticosteroids was 68%. Response rates to IVIgs, cyclosporin, and anakinra were 50%, 80%, and 100%, respectively.

Conclusions

AOSD should be recognized as a rare cause of sepsis mimic in patients with fever of unknown origin admitted to the ICU. The diagnosis relies on a few simple clinical clues. Early intensive treatment may be discussed. IVIgs should be abandoned. Long-term prognosis is favorable.

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Title: Diagnostic and management of life-threatening Adult-Onset Still Disease: a French nationwide multicenter study and systematic literature review
Authors: Antoine Néel
Anaïs Wahbi
Benoit Tessoulin
Julien Boileau
Dorothée Carpentier
Olivier Decaux
Laurence Fardet
Guillaume Geri
Pascal Godmer
Cécile Goujard
Hervé Maisonneuve
Arnaud Mari
Jacques Pouchot
Jean-Marc Ziza
Cédric Bretonnière
Mohamed Hamidou
Publication date: 01-12-2018
Publisher: BioMed Central
Published in: Critical Care / Issue 1/2018
Electronic ISSN: 1364-8535
DOI: https://doi.org/10.1186/s13054-018-2012-2

Keynote webinar | Spotlight on sleep in brain health

Springer Medicine

Diagnostic and management of life-threatening Adult-Onset Still Disease: a French nationwide multicenter study and systematic literature review

Abstract

Background

Methods

Results

Conclusions

Keynote webinar | Spotlight on sleep in brain health

Springer Medicine

Abstract

Background

Methods

Results

Conclusions

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