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
DOI: 10.2340/00015555-3673

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.

Paraneoplastic autoimmune multiorgan syndrome is a rare autoimmune bullous disease with complex features and fatal prognosis. We retrospectively studied patients with paraneoplastic autoimmune multiorgan syndrome from Peking University First Hospital (Beijing, China). Of 145 patients enrolled, Castleman disease was the most common tumour (56%), followed by thymoma (19%). Patients with Castleman disease had higher incidence of lichen planus-like lesions and bronchiolitis obliterans. The one-, three-, and five-year survival rates were 84%, 65%, and 54%, respectively. Respiratory failure (38%) and infection (22%) were the most common causes of death. Patients with the age of > 42 years and skin lesions > 17.5% body surface area may have a worse prognosis.

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