Paraneoplastic pemphigus (PNP), also known as paraneoplastic autoimmune multiorgan syndrome (PAMS), is an autoimmune blistering disease that involves the skin, mucous membranes, and multiple organs, with a high mortality rate. However, due to the rarity of PNP/PAMS, there is a lack of large-scale studies, and its clinical features and prognostic factors are not fully understood. Thus, we conducted a search in four databases: PubMed, Web of Science, EMBASE, and Scopus, and identified 290 relevant articles (a total of 504 patients). Through analysis, we summarized the demographic information, clinical manifestations, histopathology, immunological characteristics, associated tumors, treatment medications, and their survival outcomes. After drawing the Kaplan-Meier survival curves for 281 patients with available survival information, it was found that older age, circulating bullous pemphigoid 230 autoantibodies, non-Hodgkin lymphoma, and possible history of causative drugs were associated with shorter survival time. Initial oral mucosal involvement, lichenoid/interface dermatitis, Castleman disease, and epithelial-derived tumors were associated with longer survival time. In the multifactorial Cox proportional hazards regression model, non-Hodgkin lymphoma (hazard ratio, 1.959; 95% CI, 1.286-2.985; P = .002) and lichenoid/interface dermatitis (hazard ratio, 0.555; 95% CI, 0.362-0.850; P = .007) remained associated with the prognosis of PNP/PAMS patients.

中文翻译：

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副肿瘤性天疱疮和副肿瘤性自身免疫性多器官综合征的系统评价：临床特征和预后因素


副肿瘤性天疱疮 （PNP），也称为副肿瘤性自身免疫性多器官综合征 （PAMS），是一种累及皮肤、粘膜和多器官的自身免疫性水疱疾病，死亡率高。然而，由于 PNP/PAMS 的罕见性，缺乏大规模研究，其临床特征和预后因素尚不完全清楚。因此，我们在四个数据库中进行了检索：PubMed、Web of Science、EMBASE 和 Scopus，并确定了 290 篇相关文章（共 504 名患者）。通过分析，我们总结了人口学信息、临床表现、组织病理学、免疫学特征、相关肿瘤、治疗药物及其生存结局。在绘制 281 名具有可用生存信息的患者的 Kaplan-Meier 生存曲线后，发现年龄较大、循环大疱性类天疱疮 230 自身抗体、非霍奇金淋巴瘤和可能的致病药物史与较短的生存时间相关。初始口腔粘膜受累、苔藓样/界面性皮炎、Castleman 病和上皮衍生肿瘤与较长的生存时间相关。在多因素 Cox 比例风险回归模型中，非霍奇金淋巴瘤 （风险比，1.959;95% CI，1.286-2.985;P = .002） 和类苔藓/界面性皮炎 （风险比，0.555;95% CI，0.362-0.850;P = .007） 仍然与 PNP/PAMS 患者的预后相关。