title: A Systematic Review of Patients with Mucocutaneous and Respiratory Complications in Paraneoplastic Autoimmune Multiorgan Syndrome: Castleman's Disease is the Predominant Malignancy.
abstract: OBJECTIVE
  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.
authors.affiliation: MacNeal Hospital, Berwyn, IL, USA.
authors.forename: John
authors.initials: J
authors.lastname: Lee
authors.forename: Romi
authors.initials: R
authors.lastname: Bloom
authors.forename: Kyle T
authors.initials: KT
authors.lastname: Amber
mesh_terms: D001327:Autoimmune Diseases / Q000209:etiology*; D001989:Bronchiolitis Obliterans / Q000209:etiology*; D005871:Castleman Disease / Q000150:complications*; D006801:Humans; D009061:Mouth Mucosa; D010257:Paraneoplastic Syndromes / Q000209:etiology* / Q000276:immunology; D012872:Skin Diseases, Vesiculobullous / Q000209:etiology*