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

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%

SUBMITTER: Neel A 

PROVIDER: S-EPMC5896069 | biostudies-literature | 2018 Apr

REPOSITORIES: biostudies-literature