Email updates

Keep up to date with the latest news and content from BMC Pulmonary Medicine and BioMed Central.

Open Access Research article

Increased incidence of autoimmune markers in patients with combined pulmonary fibrosis and emphysema

Argyris Tzouvelekis1, George Zacharis1, Anastasia Oikonomou2, Dimitrios Mikroulis3, George Margaritopoulos4, Anastasios Koutsopoulos5, Antonis Antoniadis6, Andreas Koulelidis1, Paschalis Steiropoulos1, Panagiotis Boglou1, Matina Bakali7, Marios Froudarakis1 and Demosthenes Bouros1*

Author Affiliations

1 Department of Pneumonology, University Hospital of Alexandroupolis, Democritus University of Thrace, Alexandroupolis 68100, Greece

2 Department of Radiology, University Hospital of Alexandroupolis, Democritus University of Thrace, Alexandroupolis, Greece

3 Department of Cardiothoracic Surgery, University Hospital of Alexandroupolis, Democritus University of Thrace, Alexandroupolis, Greece

4 Department of Pneumonology, General Hospital of Kavala, Kavala, Greece

5 Department of Pathology, University Hospital of Alexandroupolis, Democritus University of Thrace, Alexandroupolis, Greece

6 Department of Pneumonology, General Hospital of Serres, Serres, Greece

7 Department of Microbiology, University Hospital of Alexandroupolis, Democritus University of Thrace, Alexandroupolis, Greece

For all author emails, please log on.

BMC Pulmonary Medicine 2013, 13:31  doi:10.1186/1471-2466-13-31

Published: 22 May 2013

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.