ArticleCritical care (London, England)2018
Diagnostic and management of life-threatening Adult-Onset Still Disease: a French nationwide multicenter study and systematic literature review.
Article in Critical care (London, England), 2018. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 38 papers, 4 of them syntheses that pooled it.
What it found
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The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
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Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
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Who cites it
38 citing papers in PubMed, 4 syntheses or guidelines pooled it, 78 citations in OpenAlex.
- Efficacy and safety of therapies for Still's disease and macrophage activation syndrome (MAS): a systematic review informing the EULAR/PReS guidelines for the management of Still's disease.Annals of the rheumatic diseases · 2024Pooled it
- EULAR/PReS recommendations for the diagnosis and management of Still's disease, comprising systemic juvenile idiopathic arthritis and adult-onset Still's disease.Annals of the rheumatic diseases · 2024Guideline
- Bibliometrics analysis on the research status and trends of adult-onset Still's disease: 1921-2021.Frontiers in immunology · 2022Pooled it
- Management of adult-onset Still's disease with interleukin-1 inhibitors: evidence- and consensus-based statements by a panel of Italian experts.Arthritis research & therapy · 2019Pooled it
- Skin biopsy in adult-onset Still's disease: A specific, rapid, and widely available diagnostic test.Journal of human immunity · 2026Article
- Review
- Unusual Cardiac Complication in Still's Disease: MAS-Induced Cardiogenic Shock.JACC. Case reports · 2026Article
- Diagnostic approach in acute hypoxemic respiratory failure.Journal of intensive medicine · 2025Review
- Shock in Adult-Onset Still's Disease Complicated by Macrophage Activation Syndrome: Clinical Characteristics and Prognosis of 14 Patients.International journal of hepatology · 2025Article
- Distribution of the causes of fever of unknown origin in China, 2013-2022.Journal of translational internal medicine · 2024Article
- Diagnosing and Treating Systemic Juvenile Idiopathic Arthritis and Adult-Onset Still's Disease as Part of the Still's Disease Continuum.Mediterranean journal of rheumatology · 2024Review
- Article
- Proposal of a new diagnostic algorithm for adult-onset Still's disease.Clinical rheumatology · 2023Article
- [Adult-onset Still's disease : Diagnosis and treatment according to the new S2e guidelines of the German Society of Rheumatology (DGRh)].Dermatologie (Heidelberg, Germany) · 2023Article
- [Adult-onset Still's disease : Diagnosis and treatment according to the new S2e guidelines of the German Society of Rheumatology (DGRh)].Zeitschrift fur Rheumatologie · 2023Article
- Diagnosis and treatment of adult-onset Still's disease: a concise summary of the German society of rheumatology S2 guideline.Zeitschrift fur Rheumatologie · 2023Review
- Clinical characteristics and outcome of elderly onset adult-onset Still's disease: A 10-year retrospective study.Journal of translational autoimmunity · 2023Article
- Adult-onset still's disease and budd-chiari syndrome: A case report.Clinical case reports · 2023Article
- Article
- Scoping review on diagnostic criteria and investigative approach in sepsis of unknown origin in critically ill patients.Journal of intensive care · 2022Article
Corrections and comments
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Authors and funding
16 authors at 12 institutions in 2 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
backgroundAdult-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.
methodsA multicenter case series of intensive care medicine (ICU) patients with life-threatening AOSD and a systematic literature review.
resultsTwenty 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.
conclusionsAOSD 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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Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.