Significance of connective tissue disease features in idiopathic interstitial pneumonia

T. J. Corte, S. J. Copley, S. R. Desai, C. J. Zappala, D. M. Hansell, A. G. Nicholson, T. V. Colby, E. Renzoni, T. M. Maher, A. U. Wells

Research output: Contribution to journalArticlepeer-review

149 Scopus citations


In idiopathic interstitial pneumonia (IIP), the significance of connective tissue disease (CTD) features in the absence of a specific CTD diagnosis remains unclear. We studied the clinical and prognostic utility of a diagnosis of undifferentiated CTD (UCTD) in patients with biopsy-proven IIP. IIP patients undergoing surgical lung biopsy (1979-2005) were studied (nonspecific interstitial pneumonia (NSIP), n=45; idiopathic pulmonary fibrosis, n=56). UCTD was considered present when serum autoantibodies were present and symptoms or signs suggested CTD. The relationship between UCTD and NSIP histology was evaluated. A clinical algorithm that best predicted NSIP histology was constructed using a priori variables. The prognostic utility of UCTD, and of this algorithm, was evaluated. UCTD was present in 14 (31%) NSIP and seven (13%) IPF patients. UCTD was not associated with a survival benefit. The algorithm predictive of NSIP (OR 10.4, 95% CI 3.21-33.67; p<0.0001) consisted of the absence of typical high-resolution computed tomography (HRCT) features for IPF and 1) a compatible demographic profile (females aged <50 yrs) or 2) Raynaud's phenomenon. In patients with an HRCT scan not typical for IPF, this algorithm predicted improved survival (hazard ratio 0.35, 95% CI 0.14-0.85; p=0.02) independent of IIP severity. UCTD is associated with NSIP histology. However, the diagnostic and prognostic significance of UCTD in IIP patients remains unclear. Copyright

Original languageEnglish (US)
Pages (from-to)661-668
Number of pages8
JournalEuropean Respiratory Journal
Issue number3
StatePublished - Mar 1 2012


  • Connective tissue disease
  • Idiopathic interstitial pneumonia
  • Idiopathic pulmonary fibrosis
  • Nonspecific interstitial pneumonia
  • Prognosis
  • Undifferentiated connective tissue disease

ASJC Scopus subject areas

  • Pulmonary and Respiratory Medicine


Dive into the research topics of 'Significance of connective tissue disease features in idiopathic interstitial pneumonia'. Together they form a unique fingerprint.

Cite this