title: Adult-onset Still disease: a rare disorder with a potentially fatal outcome.
abstract: OBJECTIVE
  The aim of this study was to assess the clinical and laboratory features of a cohort of Italian patients with adult-onset Still disease (AOSD) with particular attention on possible life-threatening complications.
  
  
  METHODS
  The clinical charts of 41 consecutive Italian patients with AOSD referred to our rheumatological department over the last 10 years were retrospectively examined. Data regarding clinical manifestations, laboratory features and complications were collected and compared with those reported in literature.
  
  
  RESULTS
  The most frequent manifestations were: fever (90.2%), arthralgias (80.4%), skin rash (75.6%), sore throat (53.6%), arthritis (51.2%), lymphadenopathy (48.7%), hepatosplenomegaly (41.4%), myalgia (21.9%), fatigue (12%), diarrhoea and vomiting (9.7%), pleural effusion (9.7%), pericardial effusion (4.8%) and abdominal pain (2.4%). In two patients whose cases are described in detail; the course of the disease was complicated by disseminated intravascular coagulopathy, in one patient with a fatal outcome. ESR, CRP and leucocyte count mean values were 69.41 mm/h, 69.05 mg/l and 18,798.5 cell/mm(3) (neutrophils 84.64%), respectively. Serum ferritin levels were increased in 48.7% of patients while transaminases were elevated in 42.6% of patients (71% considering only patients in an active phase of disease).
  
  
  CONCLUSIONS
  The results of this study are in line with those reported for other cohorts of patients. Even if the prognosis of AOSD is considered favourable, the present study indicates that the disease is a troubling condition needing prompt intervention. Occasionally, AOSD may rapidly worsen with life-threatening events.
authors.affiliation: UOC Reumatologia, Policlinico Umberto I, Università La Sapienza, Viale del Policlinico, 00161 Rome, Italy.
authors.forename: Roberta
authors.initials: R
authors.lastname: Priori
authors.affiliation: UOC Reumatologia, Policlinico Umberto I, Università La Sapienza, Viale del Policlinico, 00161 Rome, Italy.
authors.forename: Serena
authors.initials: S
authors.lastname: Colafrancesco
authors.affiliation: UOC Reumatologia, Policlinico Umberto I, Università La Sapienza, Viale del Policlinico, 00161 Rome, Italy.
authors.forename: Angelica
authors.initials: A
authors.lastname: Gattamelata
authors.affiliation: UOC Reumatologia, Policlinico Umberto I, Università La Sapienza, Viale del Policlinico, 00161 Rome, Italy.
authors.forename: Manuela
authors.initials: M
authors.lastname: Di Franco
authors.affiliation: UOC Reumatologia, Policlinico Umberto I, Università La Sapienza, Viale del Policlinico, 00161 Rome, Italy.
authors.forename: Ugo
authors.initials: U
authors.lastname: Di Tondo
authors.affiliation: UOC Reumatologia, Policlinico Umberto I, Università La Sapienza, Viale del Policlinico, 00161 Rome, Italy.
authors.forename: Guido
authors.initials: G
authors.lastname: Valesini
keywords: Adult-onset Still disease; Auto inflammatory diseases; Disseminated intravascular coagulation; Fever; Myocarditis