'The eyes see what the mind knows.' Adult-onset Still's disease, a case series and review in a south Asian population

Int J Rheum Dis. 2012 Oct;15(5):e96-100. doi: 10.1111/j.1756-185X.2012.01812.x. Epub 2012 Sep 17.

Abstract

Aim: Adult-onset Still's disease (AOSD) is a rare disease. Very few cases have been reported from the South-Asian region so the aim of this study is to assess the clinical and laboratory aspects of 15 patients with AOSD in a tertiary referral hospital in Karachi.

Methods: Retrospective data was collected from all patients diagnosed using Yamaguchi criteria for AOSD between January 2004 and December 2010 at Jinnah Medical College Hospital, Karachi.

Results: Data of 15 patients with AOSD were analyzed. Their ages ranged from 17 to 55 years, the male-to-female ratio being 6:1. The most common clinical features were fever and articular symptoms (100%), sore throat (60%), rash (53.3%), weight loss (93.3%), lymphadenopathy (40%) and elevated erythrocyte sedimentation rate (86.7%). All patients had leukocytosis with counts>20,000/mm 3 were seen in 40%. Elevated liver enzymes were present in 80% of the case series and hyperferritinemia in 100% with a mean of 3,962 ng/mL (range 555-13,865). Ambiguity in presentation and lack of serologic markers make diagnosis of AOSD difficult as 40% of patients were receiving empirical anti-tuberculous therapy prior to final diagnosis.

Conclusion: It is necessary for physicians to have a high index of suspicion for AOSD in patients with high-grade fever, arthralgia and leukocytosis.

Publication types

  • Case Reports

MeSH terms

  • Adolescent
  • Adult
  • Arthralgia / epidemiology*
  • Arthralgia / ethnology
  • Blood Sedimentation
  • Comorbidity
  • Female
  • Ferritins / blood
  • Fever / epidemiology*
  • Fever / ethnology
  • Humans
  • Leukocytosis / epidemiology*
  • Leukocytosis / ethnology
  • Male
  • Middle Aged
  • Pakistan
  • Pharyngitis / epidemiology*
  • Pharyngitis / ethnology
  • Retrospective Studies
  • Still's Disease, Adult-Onset / diagnosis*
  • Still's Disease, Adult-Onset / epidemiology*
  • Still's Disease, Adult-Onset / ethnology
  • Weight Loss
  • Young Adult

Substances

  • Ferritins