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Open Access Adult-Onset Still Disease

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We conducted a retrospective observational study to describe a cohort and identify the prognostic factors in adult-onset Still disease (AOSD). Patients enrolled in this retrospective chart review fulfilled either Yamaguchi or Fautrel criteria. Candidate variables were analyzed with logistic unadjusted and adjusted regression models.

Fifty-seven patients were seen in the internal medicine (75%) and rheumatology (25%) departments over a mean period of 8.4 years. The median time to diagnosis was 4 months. The course of AOSD was monocyclic in 17 patients, polycyclic in 25, and chronic in 15. The assessment of glycosylated ferritin (GF) in 37 patients was correlated with early diagnosis. Nine 18F-fluorodeoxyglucose positron emission tomography (18FDG-PET) scans identified the lymph nodes and glands as the main sites of hypermetabolism. Complications were frequent (n = 19), including reactive hemophagocytic syndrome (n = 8). None of the 3 deaths could be attributed to AOSD. Corticosteroid dependence, as predicted by a low GF level, occurred in 23 patients (45%). A quarter of the patients received tumor necrosis factor-α blockers or anakinra with good tolerance. Fever >39.5°C was predictive of monocyclic AOSD, while arthritis and thrombocytopenia were associated with chronic and complicated AOSD, respectively. The youngest patients had the highest risks of resistance to first-line treatments.

AOSD remains difficult to diagnose. Mortality is low despite frequent complications. GF and 18FDG-PET scans were of value in the diagnostic approach. The condition in highly symptomatic patients evolved to systemic AOSD, whereas more progressive patterns with arthritis predicted chronic AOSD.
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Keywords: 18FDG-PET = 18F-fluorodeoxyglucose positron emission tomography; AE = adverse event; AOSD = adult-onset Still disease; BM = bone marrow; CI = confidence interval; CRP = C-reactive protein; CSs = corticosteroids; CT = computed tomography; DMARDs = disease-modifying antirheumatic drugs; ESR = erythrocyte sedimentation rate; GF = glycosylated ferritin; IL = interleukin; IVIg = polyvalent intravenous immunoglobulins; MTX = methotrexate; NSAIDs = nonsteroidal antiinflammatory drugs; OR = odds ratio; PMN = polymorphonuclear neutrophils; RA = receptor antagonist; RHS = reactive hemophagocytic syndrome; SD = standard deviation; SF = serum ferritin; TNF-α = tumor necrosis factor α

Document Type: Research Article

Affiliations: 1: From the Hospices Civils de Lyon, Department of Internal Medicine (MGV, CB, PS), Croix-Rousse University Hospital, Lyon and Université Lyon I, Villeurbanne; Hospices Civils de Lyon, Service de Biostatistiques (DMB, JI), Lyon, CNRS UMR 5558, Equipe Biostatistique Santé, Pierre-Bénite, and Université Lyon I, Villeurbanne; Hospices Civils de Lyon, Department of Internal Medicine (AH, JN), Edouard Herriot University Hospital, Lyon; and Hospices Civils de Lyon, Department of Internal Medicine (ID), Centre Hospitalier Lyon Sud, Pierre-Bénite; France. 2: From the Hospices Civils de Lyon, Department of Internal Medicine (MGV, CB, PS), Croix-Rousse University Hospital, Lyon and Université Lyon I, Villeurbanne; Hospices Civils de Lyon, Service de Biostatistiques (DMB, JI), Lyon, CNRS UMR 5558, Equipe Biostatistique Santé, Pierre-Bénite, and Université Lyon I, Villeurbanne; Hospices Civils de Lyon, Department of Internal Medicine (AH, JN), Edouard Herriot University Hospital, Lyon; and Hospices Civils de Lyon, Department of Internal Medicine (ID), Centre Hospitalier Lyon Sud, Pierre-Bénite; France. 3: From the Hospices Civils de Lyon, Department of Internal Medicine (MGV, CB, PS), Croix-Rousse University Hospital, Lyon and Université Lyon I, Villeurbanne; Hospices Civils de Lyon, Service de Biostatistiques (DMB, JI), Lyon, CNRS UMR 5558, Equipe Biostatistique Santé, Pierre-Bénite, and Université Lyon I, Villeurbanne; Hospices Civils de Lyon, Department of Internal Medicine (AH, JN), Edouard Herriot University Hospital, Lyon; and Hospices Civils de Lyon, Department of Internal Medicine (ID), Centre Hospitalier Lyon Sud, Pierre-Bénite; France. 4: From the Hospices Civils de Lyon, Department of Internal Medicine (MGV, CB, PS), Croix-Rousse University Hospital, Lyon and Université Lyon I, Villeurbanne; Hospices Civils de Lyon, Service de Biostatistiques (DMB, JI), Lyon, CNRS UMR 5558, Equipe Biostatistique Santé, Pierre-Bénite, and Université Lyon I, Villeurbanne; Hospices Civils de Lyon, Department of Internal Medicine (AH, JN), Edouard Herriot University Hospital, Lyon; and Hospices Civils de Lyon, Department of Internal Medicine (ID), Centre Hospitalier Lyon Sud, Pierre-Bénite; France.

Publication date: March 1, 2014

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