A Systematic Review of Patients with Mucocutaneous and Respiratory Complications in Paraneoplastic Autoimmune Multiorgan Syndrome: Castleman's Disease is the Predominant Malignancy.

Abstract

Paraneoplastic autoimmune multiorgan syndrome (PAMS) is a rare disorder that manifests with severe mucocutaneous blistering and erosions accompanied by underlying malignancy. Constrictive bronchiolitis (CB) can occur in a subset of patients. We sought to characterize the particular group of PAMS patients with CB by comparing the underlying tumor in these patients versus all reported PAMS patients. We performed a systematic review of the Medline/Pubmed and compared the incidence of the most frequent tumor type in the CB cohort with the previously reported incidences of tumors in all documented PAMS patients. There is an increased occurrence of Castleman's tumor in patients with mucocutaneous and pulmonary complications of PAMS OR 3.86, 95% CI [2.2-6.7]. As PAMS is a syndrome with a heterogeneous presentation and autoantibody profile, the particular pathogenic pathway seen in Castleman's tumor may provide additional insight into the antigenic sites involved in PAMS-related CB.