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-te...
| Main Authors: | , , , , , , , , , , , , , , , |
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| Format: | Article |
| Language: | English |
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BMC
2018-04-01
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| Series: | Critical Care |
| Subjects: | |
| Online Access: | http://link.springer.com/article/10.1186/s13054-018-2012-2 |
| _version_ | 1818932929288470528 |
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| author | 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 |
| author_facet | 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 |
| author_sort | Antoine Néel |
| collection | DOAJ |
| description | 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. |
| first_indexed | 2024-12-20T04:40:17Z |
| format | Article |
| id | doaj.art-33e1f8b9e8854d979775e3e58acc7295 |
| institution | Directory Open Access Journal |
| issn | 1364-8535 |
| language | English |
| last_indexed | 2024-12-20T04:40:17Z |
| publishDate | 2018-04-01 |
| publisher | BMC |
| record_format | Article |
| series | Critical Care |
| spelling | doaj.art-33e1f8b9e8854d979775e3e58acc72952022-12-21T19:53:09ZengBMCCritical Care1364-85352018-04-0122111010.1186/s13054-018-2012-2Diagnostic and management of life-threatening Adult-Onset Still Disease: a French nationwide multicenter study and systematic literature reviewAntoine Néel0Anaïs Wahbi1Benoit Tessoulin2Julien Boileau3Dorothée Carpentier4Olivier Decaux5Laurence Fardet6Guillaume Geri7Pascal Godmer8Cécile Goujard9Hervé Maisonneuve10Arnaud Mari11Jacques Pouchot12Jean-Marc Ziza13Cédric Bretonnière14Mohamed Hamidou15Service de Médecine Interne, PHU3, CHU Hôtel-DieuService de Médecine Interne, PHU3, CHU Hôtel-DieuService d’Hématologie, PHU1, CHU Hôtel-DieuService de Médecine, CH de MorlaixService de Réanimation Médicale, CHU de RouenService de Médecine Interne, CHU de RennesService de Dermatologie, Hôpital Henri MondorService de Réanimation Médicale, CHU Cochin, AP-HPCH Bretagne-AtlantiqueService de Médecine Interne, CHU Bicêtre, AP-HPService de Médecine Interne, CHD VendéeService de Réanimation, Hôpital Yves Le FollService de Médecine Interne, Hôpital Européen Georges Pompidou, AP-HPService de Médecine Interne-Rhumatologie, groupe hospitalier Diaconesses-Croix-Saint-SimonService de Réanimation Médicale, PHU3, CHU de NantesService de Médecine Interne, PHU3, CHU Hôtel-DieuAbstract 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.http://link.springer.com/article/10.1186/s13054-018-2012-2Adult onset Still diseaseReactive hemophagocytic syndromeShockDifferential diagnosisAnakinraCyclosporin |
| spellingShingle | 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 Diagnostic and management of life-threatening Adult-Onset Still Disease: a French nationwide multicenter study and systematic literature review Critical Care Adult onset Still disease Reactive hemophagocytic syndrome Shock Differential diagnosis Anakinra Cyclosporin |
| title | Diagnostic and management of life-threatening Adult-Onset Still Disease: a French nationwide multicenter study and systematic literature review |
| title_full | Diagnostic and management of life-threatening Adult-Onset Still Disease: a French nationwide multicenter study and systematic literature review |
| title_fullStr | Diagnostic and management of life-threatening Adult-Onset Still Disease: a French nationwide multicenter study and systematic literature review |
| title_full_unstemmed | Diagnostic and management of life-threatening Adult-Onset Still Disease: a French nationwide multicenter study and systematic literature review |
| title_short | Diagnostic and management of life-threatening Adult-Onset Still Disease: a French nationwide multicenter study and systematic literature review |
| title_sort | diagnostic and management of life threatening adult onset still disease a french nationwide multicenter study and systematic literature review |
| topic | Adult onset Still disease Reactive hemophagocytic syndrome Shock Differential diagnosis Anakinra Cyclosporin |
| url | http://link.springer.com/article/10.1186/s13054-018-2012-2 |
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