Lung disease with anti-CCP antibodies but not rheumatoid arthritis or connective tissue disease

Abstract

Objective: We sought to characterize a novel cohort of patients with lung disease, anti-cyclic citrullinated peptide (CCP) antibody positivity, without rheumatoid arthritis (RA) or other connective tissue disease (CTD).

Methods: The study sample included 74 subjects with respiratory symptoms, evaluated January 2008-January 2010 and found to have a positive anti-CCP antibody but no evidence for RA or other CTD. Each underwent serologic testing, pulmonary physiology testing, and thoracic high-resolution computed tomography (HRCT) scan as part of routine clinical evaluation.

Results: The majority of subjects were women, and most were former cigarette smokers. Four distinct radiographic phenotypes were identified: isolated airways disease (54%), isolated interstitial lung disease (ILD) (14%), mixed airways disease and ILD (26%), and combined pulmonary fibrosis with emphysema (7%). This cohort had a predominance of airways disease, either in isolation or along with a usual interstitial pneumonia-pattern of ILD. Among subjects with high-titer anti-CCP positivity (n=33), three developed the articular manifestations of RA during a median follow-up of 449 days.

Conclusion: We have described a unique cohort of patients with anti-CCP antibody positivity and lung disease in the absence of existing RA or other CTD. The lung phenotypic characteristics of this cohort resemble those of established RA and a few of these patients have developed articular RA within a short period of follow-up. The implications of a positive anti-CCP antibody among patients with lung disease but not RA are not yet known, but we believe requires further investigation.

Conflict of interest statement

Conflict of interest

None of the authors have any conflicts of interest pertinent to this research to disclose.

Copyright © 2012 Elsevier Ltd. All rights reserved.

Figures

Figure 1

Photomicrograph (10×) of histopathology from transbronchial biopsy demonstrating airways disease with lymphoplasmacytic inflammation in a subject with anti-CCP positivity without RA.

Figure 2

Photomicrograph (20×) of histopathology from surgical lung biopsy demonstrating UIP with lymphoid aggregates and germinal center formation in a subject with anti-CCP positivity without RA.