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Abstract: SA-PO0810

Baseline Characteristics and Treatment Patterns in Chinese Patients with Atypical Hemolytic Uremic Syndrome (aHUS): Preliminary Results from a Prospective, Observational Study

Session Information

Category: Glomerular Diseases

  • 1402 Glomerular Diseases: Clinical, Outcomes, and Therapeutics

Authors

  • Tan, Ying, Peking University First Hospital, Beijing, Beijing, China
  • Liu, Xiaorong, Beijing Children's Hospital Capital Medical University, Beijing, Beijing, China
  • Li, Ning, Second Hospital of Shanxi Medical University, Taiyuan, Shanxi, China
  • Lu, Wanhong, The First Affiliated Hospital of Xi'an Jiaotong University, Xi'an, Shaanxi, China
  • Chen, Wei, The First Affiliated Hospital of Sun Yat-sen University, Guangzhou, Guangdong, China
  • Fu, Ping, West China Hospital of Sichuan University, Chengdu, Sichuan, China
  • Xing, Guangqun, The Affiliated Hospital of Qingdao University, Qingdao, Shandong, China
  • Yin, Lei, Shanghai Children's Medical Center Affiliated to Shanghai Jiaotong University School of Medicine, Shanghai, Shanghai, China
  • Zhao, Fei, Children's Hospital of Nanjing Medical University, Nanjing, Jiangsu, China
  • Li, Guisen, Sichuan Academy of Medical Sciences and Sichuan People's Hospital, Chengdu, Sichuan, China
  • Luo, Rongmu, Aerospace Science and Industry Corporation 731 hospital, Beijing, Beijing, China
  • Xu, Hui, Xiangya Hospital Central South University, Changsha, Hunan, China
  • Zhao, Ming-Hui, Peking University First Hospital, Beijing, Beijing, China
Background

aHUS is a rare, potentially life-threatening disease due to complement dysregulation and is clinically diagnosed by excluding other causes of thrombotic microangiopathies (TMA). With limited research in Chinese aHUS patients (pts) and the introduction of eculizumab since 2022, this ongoing study aims to better characterise this population.

Methods

The Aurora study (NCT06099236) is a prospective, observational study that is enrolling pts of any age with diagnosed aHUS from 60 sites. This analysis assessed demographics, baseline disease characteristics and treatment patterns.

Results

163 aHUS pts were enrolled between 15 Jan 2024 to 15 Jan 2025, with 130 (79.8%) receiving eculizumab (mean initiation time 16.6 d). See Table for detailed characteristics. 16 pts had a history of relapse, including 4 without and 6 with genetic or acquired abnormalities. 119 pts (73.0%) had triggers, with the top 3 being infection (29.4%), autoimmune disease (24.4%), and malignant hypertension (12.6%). 39 of 65 pts that received kidney biopsy had confirmed TMA. Only 44.6% and 63.6% pts had genetic or acquired/serum complement abnormalities, respectively.

Conclusion

This is the first and largest study to show China-specific aHUS characteristics. The types of triggers most reported suggest a unique pattern among this population. The data suggest a limited role for serum complement testing and histological findings in diagnosis and show wide adoption of eculizumab as 1st-line therapy with early initiation in China. The occurrence of relapses regardless of genetic status indicates the need for standardized management in all aHUS pts.

Table

Funding

  • Commercial Support – AstraZeneca China

Digital Object Identifier (DOI)