Risk of macrophage activation syndrome in patients with adult-onset Still's disease with skin involvement: A retrospective cohort study

Background: Small case series and case reports indicated that atypical persistent pruritic eruptions (PPEs), another type of skin lesions seen in adult-onset Still's disease (AOSD), imply a worse prognosis than typical evanescent rashes. Objective: To investigate clinical characteristics and macrophage activation syndrome (MAS) occurrence in AOSD with PPEs. Methods: A retrospective cohort study analyzed 150 patients with AOSD with rashes at the First Affiliated Hospital of Zhejiang University from January 2013 to December 2019. Results: Patients with AOSD with PPEs had higher lactate dehydrogenase (492.00 U/L vs 382.00 U/L; P < .001) and ferritin (6944.10 ng/ml vs 4286.60 ng/ml; P = .033), and lower fibrinogen (5.05 g/L vs 5.77 g/ L; P = .014) than those with evanescent rashes. Patients with AOSD with PPEs had a higher incidence (17.4% vs 3.1%, P = .006) and cumulative event rate for MAS (P = .008) and tended to receive high-dose glucocorticoid (36% vs 20.3%; P = .036). Multivariate analysis indicated that PPEs (hazard ratio [HR], 5.519; 95% confidence interval [CI], 1.138-26.767; P = .034), aspartate aminotransferase of greater than 120 U/L (HR, 8.084; 95% CI, 1.728-37.826; P = .008), and splenomegaly (HR, 21.152; 95% CI, 2.263-197.711; P = .007) were independent risk factors for MAS. Limitations: Single-center, retrospective nature, small sample size. Conclusion: PPEs indicated increased severity and MAS occurrence versus evanescent rashes. PPEs, aspartate aminotransferase of greater than 120 U/L, and splenomegaly were risk factors for MAS in AOSD with skin involvement.

Automated summary

Patients with AOSD with PPEs showed higher levels of lactate dehydrogenase and ferritin, lower levels of fibrinogen, and had a higher incidence and cumulative event rate for MAS compared to those with evanescent rashes. PPEs, elevated aspartate aminotransferase levels, and splenomegaly were independently associated with an increased risk of MAS in AOSD patients with skin involvement.