title: Increased incidence of autoimmune markers in patients with combined pulmonary fibrosis and emphysema.
abstract: BACKGROUND
  Combined pulmonary fibrosis and emphysema (CPFE) is an umbrella term encompassing upper lobe emphysema and lower lobe pulmonary fibrosis with pathogenesis elusive. The aim of our study was to investigate the incidence of autoimmune markers in patients with CPFE.
  
  
  METHODS
  In this multicenter study we retrospectively evaluated records from patients with CPFE (n=40) and IPF (n=60) without emphysema. Baseline demographic characteristics, high-resolution computed tomography (HRCT), spirometry, histopathological, treatment, serum immunologic and survival data were investigated. B cell presence was estimated with CD20 immunostaining in representative lung biopsy samples from CPFE patients and control subjects.
  
  
  RESULTS
  A statistically significant increased number of CPFE patients with elevated serum ANA with or without positive p-ANCA titers compared to patients with IPF without emphysema was observed. Patients with CPFE and positive autoimmune markers exhibited improved survival compared to patients with a negative autoimmune profile. A massive infiltration of clusters of CD20+ B cells forming lymphoid follicles within the fibrotic lung in CPFE patients with positive serum immunologic profile compared to patients with negative profile, was noted and positively correlated with improved survival.
  
  
  CONCLUSIONS
  A significant proportion of patients with CPFE may present with underlying auto-immune disorders that may reside insidiously and be associated with favorable prognosis. Early identification of these patients using a panel of auto-antibodies may lead to more targeted and effective therapeutic applications.
authors.affiliation: Department of Pneumonology, University Hospital of Alexandroupolis, Democritus University of Thrace, Alexandroupolis 68100, Greece.
authors.forename: Argyris
authors.initials: A
authors.lastname: Tzouvelekis
authors.forename: George
authors.initials: G
authors.lastname: Zacharis
authors.forename: Anastasia
authors.initials: A
authors.lastname: Oikonomou
authors.forename: Dimitrios
authors.initials: D
authors.lastname: Mikroulis
authors.forename: George
authors.initials: G
authors.lastname: Margaritopoulos
authors.forename: Anastasios
authors.initials: A
authors.lastname: Koutsopoulos
authors.forename: Antonis
authors.initials: A
authors.lastname: Antoniadis
authors.forename: Andreas
authors.initials: A
authors.lastname: Koulelidis
authors.forename: Paschalis
authors.initials: P
authors.lastname: Steiropoulos
authors.forename: Panagiotis
authors.initials: P
authors.lastname: Boglou
authors.forename: Matina
authors.initials: M
authors.lastname: Bakali
authors.forename: Marios
authors.initials: M
authors.lastname: Froudarakis
authors.forename: Demosthenes
authors.initials: D
authors.lastname: Bouros
mesh_terms: D000328:Adult; D000368:Aged; D000888:Antibodies, Anti-Idiotypic / Q000097:blood; D019268:Antibodies, Antineutrophil Cytoplasmic / Q000097:blood; D018951:Antigens, CD20 / Q000378:metabolism; D015551:Autoimmunity / Q000502:physiology*; D015415:Biomarkers / Q000097:blood; D001706:Biopsy; D016022:Case-Control Studies; D015897:Comorbidity; D004646:Emphysema / Q000453:epidemiology* / Q000276:immunology* / Q000401:mortality; D005260:Female; D006801:Humans; D053208:Kaplan-Meier Estimate; D008168:Lung / Q000378:metabolism / Q000473:pathology; D008297:Male; D008875:Middle Aged; D011658:Pulmonary Fibrosis / Q000453:epidemiology* / Q000276:immunology* / Q000401:mortality; D012189:Retrospective Studies
chemical_list: D000888:Antibodies, Anti-Idiotypic; D019268:Antibodies, Antineutrophil Cytoplasmic; D018951:Antigens, CD20; D015415:Biomarkers