RT Journal Article
SR Electronic
T1 Significance of Connective Tissue Disease features in Idiopathic Interstitial Pneumonia
JF European Respiratory Journal
JO Eur Respir J
FD European Respiratory Society
SP erj01749-2010
DO 10.1183/09031936.00174910
A1 T.J. Corte
A1 S.J. Copley
A1 S.R. Desai
A1 C.J. Zappala
A1 D.M. Hansell
A1 A.G. Nicholson
A1 T.V. Colby
A1 E. Renzoni
A1 T.M. Maher
A1 A.U. Wells
YR 2011
UL http://erj.ersjournals.com/content/early/2011/09/20/09031936.00174910.abstract
AB In idiopathic interstitial pneumonia (IIP), the significance of connective tissue disease (CTD) features in the absence of a specific CTD diagnosis remains unclear. We study the clinical and prognostic utility of a diagnosis of UCTD in patients with biopsy-proven idiopathic interstitial pneumonia (IIP). IIP patients undergoing surgical lung biopsy (1979–2005) were studied (NSIP, n=45; idiopathic pulmonary fibrosis, IPF, n=56). UCTD was considered present when serum auto-antibodies were positive and symptoms or signs suggested CTD. The relationship between UCTD and NSIP histology was evaluated. A clinical algorithm, which 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 7(13%) IPF patients. UCTD was not associated with a survival benefit. The algorithm predictive of NSIP (OR 10.4; CI 3.21, 33.67; p<0.0001) consisted of the absence of typical HRCT features for IPF, and a) compatible demographic profile (females aged <50yrs) or b) Raynaud's phenomenon. In patients with atypical HRCT, this algorithm predicted improved survival (HR 0.35; 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.