A systematic review of paraneoplastic pemphigus and paraneoplastic autoimmune multiorgan syndrome: Clinical features and prognostic factors

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.