Adult-Onset Still Disease

• DOI: 10.1097/md.0000000000000021
• Publication Date: 2014-03-03T20:49:22Z
• Authors: Mathieu Gerfaud-V...; Delphine Maucort-...; Arnaud Hot; Jean Iwaz; Jacques Ninet; Isabelle Durieu; Christiane Brouss...; Pascal Sève
• Keywords: Adult; Male; Adolescent; [SDV]Life Sciences [q-bio]; Middle Aged; Prognosis; 3. Good health; Young Adult; 03 medical and health sciences; 0302 clinical medicine; Fluorodeoxyglucose F18; Risk Factors; Antirheumatic Agents; Positron-Emission Tomography; Ferritins; Humans; Original Study; Female; Radiopharmaceuticals; Still's Disease, Adult-Onset; Aged; Retrospective Studies

We conducted a retrospective observational study to describe cohort and identify the prognostic factors in adult-onset Still disease (AOSD). Patients enrolled this chart review fulfilled either Yamaguchi or Fautrel criteria. Candidate variables were analyzed with logistic unadjusted adjusted regression models. Fifty-seven patients seen internal medicine (75%) rheumatology (25%) departments over mean period of 8.4 years. The median time diagnosis was 4 months. course AOSD monocyclic 17 patients, polycyclic 25, chronic 15. assessment glycosylated ferritin (GF) 37 correlated early diagnosis. Nine 18F-fluorodeoxyglucose positron emission tomography (18FDG-PET) scans identified lymph nodes glands as main sites hypermetabolism. Complications frequent (n = 19), including reactive hemophagocytic syndrome 8). None 3 deaths could be attributed AOSD. Corticosteroid dependence, predicted by low GF level, occurred 23 (45%). A quarter received tumor necrosis factor-α blockers anakinra good tolerance. Fever >39.5°C predictive AOSD, while arthritis thrombocytopenia associated complicated respectively. youngest had highest risks resistance first-line treatments. remains difficult diagnose. Mortality is despite complications. 18FDG-PET value diagnostic approach. condition highly symptomatic evolved systemic whereas more progressive patterns