Features and Risk Factors for Paraneoplastic Autoimmune Multiorgan Syndrome in 145 Chinese Patients

• Main Authors: Mingyue Wang, Furong Li, Xintong Wang, Xue Wang, Rui Wang, Yinmo Yang, Jian Li, Shijie Zhang, Weiming Huang, Yujun Dong, Xiangdong Mu, Ting Li, Kaiwen Ni, Xixue Chen, Xuejun Zhu
• Format: Article
• Language: English
• Published: Society for Publication of Acta Dermato-Venereologica 2020-11-01
• Series: Acta Dermato-Venereologica
• Subjects: paraneoplastic autoimmune multiorgan syndrome; castleman disease; bronchiolitis obliterans; survival rate; risk factors
• Online Access: https://www.medicaljournals.se/acta/content/html/10.2340/00015555-3673
• ISSN: 0001-5555; 1651-2057

Paraneoplastic autoimmune multiorgan syndrome is a complex and deadly disease. We retrospectively reviewed the clinical features and risk factors for paraneoplastic autoimmune multiorgan syndrome in 145 Chinese patients. The most common neoplasm was Castleman disease (56%), and patients with Castleman disease tended to be younger (≤ 42 years old: 83% vs. 29%) and to have a greater proportions of lichen planus-like lesions (47% vs. 27%) and bronchiolitis obliterans (49% vs. 29%), compared to other neoplasm-associated patients. Among all 145 patients in the study, the survival rates were 84% at 1 year, 65% at 3 years, and 54% at 5 years. Kaplan-Meier curve analysis revealed that mortality was associated with older age (> 42 years), neoplasm type, labial lesions, and larger skin lesion area (> 17.5% of the body surface area). However, only older age and larger skin lesion area were independent factors associated with mortality in multivariate analysis. We suggest that patients with Castleman disease and paraneoplastic autoimmune multiorgan syndrome have many unique characteristics and the underlying risk factors for death require further exploration.