Clinical Spectrum of Adult-Onset Still’s Disease in Patients Presenting At A Tertiary Care Hospital in Pakistan: A 16-Year Follow-Up Study

Abstract

Objectives Adult-onset Still’s Disease (AOSD) is a rare systemic auto-inflammatory disorder with a low incidence rate and limited data. This study aims to lay down the clinical and laboratory spectrum, associated complications, and outcomes of patients who presented to a tertiary care center – in order to identify any regional variation in the presentation and enable physicians to develop a thought process in patients without characteristic features. Materials and Methods We reviewed the records of patients from January 2005 - February 2021 who presented with symptoms consistent with AOSD, using the Yamaguchi criteria. The patients’ clinical symptoms, laboratory values, therapeutic management, complications, and outcomes were analysed. Descriptive statistics were produced to illustrate the pattern of the disease. Results The mean age of patients was 37.7 years, with a male to female ratio of 1:1. Fever and arthralgia was seen in 90%, a salmon-coloured rash in 60%, and leukocytosis in 80%. 30% presented with a sore throat and recent lymphadenopathy, 40% with hepatomegaly and/or splenomegaly on imaging, 100% with abnormal liver function tests. Steroids were used as first-line treatment. Most patients did not present with common, previously reported, complications of AOSD. Conclusions The clinical aspects in this study are, to a large part, comparable to those seen in other parts of the world. Thus, a comprehensive analysis of AOSD would enable physicians to broaden their minds and keep it as a top differential in atypical presentations – enhancing patient care by preventing a delay in diagnosis and initiation of prompt management.