title: 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.
  
  
  CONCLUSIONS
  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.
authors.affiliation: Autoimmune Lung Center, National Jewish Health, Denver, CO 80206, USA. fischera@njhealth.org
authors.forename: Aryeh
authors.initials: A
authors.lastname: Fischer
authors.forename: Joshua J
authors.initials: JJ
authors.lastname: Solomon
authors.forename: Roland M
authors.initials: RM
authors.lastname: du Bois
authors.forename: Kevin D
authors.initials: KD
authors.lastname: Deane
authors.forename: Amy L
authors.initials: AL
authors.lastname: Olson
authors.forename: Evans R
authors.initials: ER
authors.lastname: Fernandez-Perez
authors.forename: Tristan J
authors.initials: TJ
authors.lastname: Huie
authors.forename: Allen D
authors.initials: AD
authors.lastname: Stevens
authors.forename: Mary B
authors.initials: MB
authors.lastname: Gill
authors.forename: Avi M
authors.initials: AM
authors.lastname: Rabinovitch
authors.forename: David A
authors.initials: DA
authors.lastname: Lynch
authors.forename: David A
authors.initials: DA
authors.lastname: Burns
authors.forename: Isabel S
authors.initials: IS
authors.lastname: Pineiro
authors.forename: Steve D
authors.initials: SD
authors.lastname: Groshong
authors.forename: Rosane D
authors.initials: RD
authors.lastname: Duarte Achcar
authors.forename: Kevin K
authors.initials: KK
authors.lastname: Brown
authors.forename: Richard J
authors.initials: RJ
authors.lastname: Martin
authors.forename: Jeffrey J
authors.initials: JJ
authors.lastname: Swigris
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D001172:Arthritis, Rheumatoid / Q000276:immunology*; D001323:Autoantibodies / Q000097:blood*; D001982:Bronchial Diseases / Q000276:immunology* / Q000473:pathology / Q000503:physiopathology; D003240:Connective Tissue Diseases / Q000276:immunology; D005260:Female; D006801:Humans; D017563:Lung Diseases, Interstitial / Q000276:immunology* / Q000473:pathology / Q000503:physiopathology; D008297:Male; D008875:Middle Aged; D010456:Peptides, Cyclic / Q000276:immunology*; D011446:Prospective Studies; D012129:Respiratory Function Tests; D012189:Retrospective Studies; D014057:Tomography, X-Ray Computed / Q000379:methods; D055815:Young Adult
chemical_list: D001323:Autoantibodies; D010456:Peptides, Cyclic; C487763:cyclic citrullinated peptide