Manifestations, Outcomes of Adult Onset Still Disease

Individuals with adult onset Still disease (AOSD) may present with elevated inflammatory markers, leukocytosis, and anemia, according to the results of a retrospective study presented at American College of Rheumatology Convergence 2022.

The researchers examined patient demographics, objective lab findings, and outcomes of 185 patients with AOSD who were aged 18 years or older. The International Classification of Diseases (10th revision) code M06.1 was utilized to identify patients who were diagnosed with AOSD at one of 185 hospitals from 2016 to 2021. 

The results indicated that the incidence rate of AOSD was 0.16 to 0.21 per 100,000 people. Participants had a mean age of 47 years and almost 70% of participants were women. The overall mortality rate was 3.2%.

Among the elevated inflammatory markers observed in these patients were erythrocyte sedimentation rate (55.111 +/- 36.52), c-reactive protein (12.26 +/- 27.48), and ferritin (71411.23 +/- 11536.60). Additional presentations included leukocytosis (12.15 +/- 5.86) and unspecified anemia (23%).

Further, approximately 24% of patients required admission to the intensive care unit (ICU). Coexisting pneumonia was present in 11.9% of patients. This was associated with an increase in ICU admission (likelihood ratio 7.4, p = .007) but was not associated with an increase in mortality.

“Our study establishes patient demographics and common objective findings to better understand and diagnose this rare disease,” the authors concluded. “Incidence rates increased over time, suggesting increased awareness and improved diagnosis.”


—Leigh Precopio



Bui C, Collins T, Venkat K, Ayutyanont N, Vickery K, Kubomoto S. Adult onset still’s disease: a retrospective study of objective manifestations and outcomes. Paper presented at: American College of Rheumatology Convergence 2022; November 10-14, 2022; Philadelphia, PA. Accessed November 2, 2022.