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Title: A Systematic Review of Patients with Mucocutaneous and Respiratory Complications in Paraneoplastic Autoimmune Multiorgan Syndrome: Castleman's Disease is the Predominant Malignancy. Author: Lee J, Bloom R, Amber KT. Journal: Lung; 2015 Aug; 193(4):593-6. PubMed ID: 25903794. Abstract: PURPOSE: 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. METHODS: 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. RESULTS: 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]. CONCLUSIONS: 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.[Abstract] [Full Text] [Related] [New Search]