OBJECTIVE: Adult-onset Still's disease (AOSD) is a systemic inflammatory disorder of unknown etiology, characterized by high fever, transient cutaneous rash, arthralgia/arthritis, and leukocytosis. Liver involvement in AOSD has been described, but few reports have described it in depth. The present study analyzed clinical and laboratory features in a series of Chinese AOSD patients. METHODS: Data of 77 patients with AOSD (fulfilling Yamaguchi's diagnostic criteria) were retrospectively reviewed and compared with other series. RESULTS: The characteristics of our patients are similar to those reported in the literature. Hepatomegaly occurred in 11.7% of the cases; abnormal liver enzymes in 62.3% mild cytolysis (level of transaminases <2 N) (23.4%), moderate cytolysis (between 2 and 5 N) (23.4%), severe cytolysis (>5 N) (15.6%), and increase in the level of alkaline phosphatase (32.9%), gamma-glutamyltransferase (48.1%), lactic dehydrogenase (69.0%). Complete recovery occurred in all patients, except for 1 who died of severe liver failure and complications. CONCLUSION: AOSD is a systemic disease, and the present study reemphasizes the high frequency of liver involvement. Although it was slight to moderate in most cases, severe cytolytic hepatitis has been described. Treatment for AOSD patients with liver involvement aimed mainly at AOSD itself and most of the patients with liver involvement got complete remission with systemic corticosteroid therapy.
OBJECTIVE: Adult-onset Still's disease (AOSD) is a systemic inflammatory disorder of unknown etiology, characterized by high fever, transient cutaneous rash, arthralgia/arthritis, and leukocytosis. Liver involvement in AOSD has been described, but few reports have described it in depth. The present study analyzed clinical and laboratory features in a series of Chinese AOSD patients. METHODS: Data of 77 patients with AOSD (fulfilling Yamaguchi's diagnostic criteria) were retrospectively reviewed and compared with other series. RESULTS: The characteristics of our patients are similar to those reported in the literature. Hepatomegaly occurred in 11.7% of the cases; abnormal liver enzymes in 62.3% mild cytolysis (level of transaminases <2 N) (23.4%), moderate cytolysis (between 2 and 5 N) (23.4%), severe cytolysis (>5 N) (15.6%), and increase in the level of alkaline phosphatase (32.9%), gamma-glutamyltransferase (48.1%), lactic dehydrogenase (69.0%). Complete recovery occurred in all patients, except for 1 who died of severe liver failure and complications. CONCLUSION: AOSD is a systemic disease, and the present study reemphasizes the high frequency of liver involvement. Although it was slight to moderate in most cases, severe cytolytic hepatitis has been described. Treatment for AOSD patients with liver involvement aimed mainly at AOSD itself and most of the patients with liver involvement got complete remission with systemic corticosteroid therapy.
Authors: Mathieu Gerfaud-Valentin; Pascal Sève; Jean Iwaz; Anne Gagnard; Christiane Broussolle; Isabelle Durieu; Jacques Ninet; Arnaud Hot Journal: Medicine (Baltimore) Date: 2014-10 Impact factor: 1.889