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

1, 2, 3, 4, 5, 6, 7, 8, 9, 10, 11, 12, 13, 14, 15, 16, 17, 18, 19, 20, 21, 22, 23, 24

 

  1. Department of Rheumatology and Immunology, Shanghai Jiao Tong University School of Medicine Affiliated Ruijin Hospital, Shanghai, China.
  2. Xinhua Hospital Chongming Branch Affiliated to Shanghai JiaoTong University School of Medicine, Shanghai, China.
  3. Department of Rheumatology, First Affiliated Hospital of the Medical School, Zhejiang University, Hangzhou, China.
  4. Department of Rheumatology and Immunology, People's Hospital of Xinjiang Ugyur Autonomous Region, Urumqi, China.
  5. Department of Rheumatology and Immunology, First Affiliated Hospital of Kunming Medical University, Kunming, China.
  6. Department of Rheumatology and Immunology, First Affiliated Hospital of Bengbu Medical College, China.
  7. Department of Rheumatology, Zhongshan Hospital, Fudan University, Shanghai, China.
  8. Shanghai Jiao Tong University School of Medicine, Shanghai, China.
  9. Department of Rheumatology and Immunology, People's Hospital of Xinjiang Ugyur Autonomous Region, Urumqi, China.
  10. Department of Rheumatology and Immunology, Shanghai Jiao Tong University School of Medicine Affiliated Ruijin Hospital, Shanghai, China.
  11. Department of Rheumatology and Immunology, Shanghai Jiao Tong University School of Medicine Affiliated Ruijin Hospital, Shanghai, China.
  12. Department of Rheumatology and Immunology, Shanghai Jiao Tong University School of Medicine Affiliated Ruijin Hospital, Shanghai, China.
  13. Department of Rheumatology and Immunology, Shanghai Jiao Tong University School of Medicine Affiliated Ruijin Hospital, Shanghai, China.
  14. Department of Rheumatology and Immunology, Shanghai Jiao Tong University School of Medicine Affiliated Ruijin Hospital, Shanghai, China.
  15. Department of Rheumatology and Immunology, Shanghai Jiao Tong University School of Medicine Affiliated Ruijin Hospital, Shanghai, China.
  16. Department of Rheumatology and Immunology, Shanghai Jiao Tong University School of Medicine Affiliated Ruijin Hospital, Shanghai, China.
  17. Department of Rheumatology and Immunology, Shanghai Jiao Tong University School of Medicine Affiliated Ruijin Hospital, Shanghai, China.
  18. Department of Rheumatology and Immunology, First Affiliated Hospital of Bengbu Medical College, China.
  19. Department of Rheumatology and Immunology, First Affiliated Hospital of Kunming Medical University, Kunming, China.
  20. Department of Rheumatology, Zhongshan Hospital, Fudan University, Shanghai, China.
  21. Department of Rheumatology and Immunology, People's Hospital of Xinjiang Ugyur Autonomous Region, Urumqi, China.
  22. Department of Rheumatology, First Affiliated Hospital of the Medical School, Zhejiang University, Hangzhou, China.
  23. Department of Rheumatology and Immunology, Shanghai Jiao Tong University School of Medicine Affiliated Ruijin Hospital, Shanghai, China. yangchengde@sina.com
  24. Department of Rheumatology and Immunology, Shanghai Jiao Tong University School of Medicine Affiliated Ruijin Hospital, Shanghai, China. hong-lei-liu@163.com

CER11835
2019 Vol.37, N°6 ,Suppl.121
PI 0052, PF 0057
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PMID: 31573475 [PubMed]

Received: 21/10/2018
Accepted : 25/03/2019
In Press: 27/09/2019
Published: 09/12/2019

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 30CONCLUSIONS:
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.

Rheumatology Article