Clinical features and prognosis of adult-onset still's disease: 61 cases from China

J Rheumatol. 2009 May;36(5):1026-31. doi: 10.3899/jrheum.080365. Epub 2009 Feb 27.

Abstract

Objective: To describe the onset, clinical features, prognostic factors, and treatment of adult-onset Still's disease (AOSD) in cases from China.

Methods: Sixty-one Chinese patients with AOSD were analyzed retrospectively.

Results: Common clinical features were fever (100.0%), rash (88.5%), and arthritis (82.0%). The laboratory findings were as follows: leukocytosis (83.6%), increased erythrocyte sedimentation rate (100.0%), elevated transaminase concentrations (23.0%), elevated ferritin levels (79.6%), negative antinuclear antibody (88.5%), and negative rheumatoid factor (88.5%). Of the 61 patients, 44.3% exhibited a monocyclic disease pattern, 29.5% experienced disease relapse at least once, 16.4% exhibited chronic articular course, and 9.8% died; most deaths were due to pulmonary infection and respiratory failure. Based on the disease course, we divided the 61 patients into 2 groups: those with favorable outcome (cyclic disease course, n = 45) and unfavorable outcome (chronic disease course or death, n = 16). We analyzed the prognostic factors for the 2 groups, and found that pleuritis, interstitial pneumonia, elevated ferritin levels, and failure of fever to subside after 3 days of prednisolone at 1 mg/kg/day were unfavorable prognostic factors for patients with AOSD.

Conclusion: Patients with AOSD had complex symptoms with no specific laboratory findings. Our results indicate that AOSD is not a relatively benign disease, especially in cases that are refractory to high doses of prednisone.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adult
  • Arthritis / etiology
  • Arthritis / pathology
  • Asian People*
  • Blood Sedimentation
  • Cause of Death
  • China / epidemiology
  • Exanthema / etiology
  • Exanthema / pathology
  • Female
  • Fever / complications
  • Fever / pathology
  • Humans
  • Leukocytosis / etiology
  • Leukocytosis / pathology
  • Male
  • Middle Aged
  • Predictive Value of Tests
  • Prednisone / therapeutic use
  • Prognosis
  • Retrospective Studies
  • Risk Factors
  • Still's Disease, Adult-Onset / complications
  • Still's Disease, Adult-Onset / drug therapy
  • Still's Disease, Adult-Onset / mortality
  • Still's Disease, Adult-Onset / pathology*
  • Survival Rate
  • Treatment Failure
  • Young Adult

Substances

  • Prednisone