Introduction: Adult-onset Still's Disease (AOSD) is a rare systemic auto-inflammatory disorder with limited available data and regional variations in presentation. This study aims to shed light on the clinical and laboratory spectrum and associated complications of AOSD patients at a tertiary care center. The goal is to facilitate early diagnosis and management in cases lacking characteristic features.
Methods: We conducted a retrospective review of patient records from January 2005 to February 2021, utilizing the Yamaguchi criteria for AOSD diagnosis. Clinical symptoms, laboratory values, therapeutic approaches and complications were analyzed. Descriptive statistics were employed to illustrate disease patterns.
Results: The study included patients with a mean age of 37.6 years, with a male-to-female ratio of 3:5. Fever and arthralgia were prevalent in 100% of cases, while a salmon-colored rash was observed in 62.5%, and leukocytosis in 75%. Additional symptoms included sore throat and recent lymphadenopathy in 37.5%, hepatomegaly and/or splenomegaly on imaging in 50%, and abnormal liver function tests in 87.5% cases. Steroids were the primary first-line treatment. Notably, most patients did not exhibit the common complications previously reported in AOSD.
Conclusion: The clinical findings in our study closely resemble those reported in other regions, emphasizing the need for a comprehensive analysis of AOSD. This study underscores the importance of considering AOSD as a potential diagnosis, even in atypical presentations, to enhance patient care by minimizing delays in diagnosis and enabling prompt management.
Key points
• This study presents the first national data on AOSD in Pakistan
• AOSD mostly affects younger adults, with a mean age of 37.6 years
• Common AOSD symptoms include fever, arthralgia, leukocytosis, high ferritin levels, and abnormal liver function, but the rash's prevalence varies by region.