Clinical features and current treatments of adult-onset Still's disease: a multicentre survey of 517 patients in China.

ABSTRACT

OBJECTIVES: As a rare systemic autoinflammatory disease, adult-onset Still's disease (AOSD) has heterogeneous clinical manifestations, response to treatment and outcome. This study tried to assess the clinical characteristics, laboratory tests, and treatments of Chinese AOSD patients, and make a retrospective analysis. METHODS: We collected from 7 hospitals in China a total of 517 Chinese patients with AOSD who satisfied the Yamaguchi criteria. We retrospectively evaluated their clinical features, laboratory tests, treatments and compared them with published data from different studies. All the data in this study were from medical records and further statistic analyses. RESULTS: We evaluated a total of 517 AOSD patients, 72% female, average age of onset was 37.7; spiking fever, rash and arthralgia occurred in 472 (91.3%), 413 (79.9%), 378 (73.1%) cases, respectively. There were 439/513 (85.6%) cases with leukocytosis and 456/476 (95.8%) cases with raised serum ferritin. The highest frequently used medications and regimens for remission were glucocorticoids (498/517, 96.3%), methotrexate (273/517, 52.8%) and hydroxychloroquine (174/517, 33.7%). 84.4%. 357/423 of AOSD cases were able to achieve initial remission with different regimens, mostly including glucocorticoids, methotrexate or hydroxychloroquine. 47.2% of them (244/517) received 30prednisone to reach final clinical remission. Further analysis indicated that risk factors, such as skin rash, pericarditis, splenomegaly and delayed diagnosis, are highly related to the dosage of prednisone for remission.

CONCLUSIONS:

Glucocorticoids are mostly selected to induce remission in China and half of them required ~0.5-1mg/kgbw prednisone. In patients with skin rash, pericarditis, splenomegaly or delayed diagnosis, a higher dosage of prednisone was needed to obtain remission.