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Significance of connective tissue disease features in idiopathic interstitial pneumonia.

Corte, TJ; Copley, SJ; Desai, SR; Zappala, CJ; Hansell, DM; Nicholson, AG; ... Wells, AU; + view all (2012) Significance of connective tissue disease features in idiopathic interstitial pneumonia. Eur Respir J , 39 (3) 661 - 668. 10.1183/09031936.00174910.

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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.

Title:Significance of connective tissue disease features in idiopathic interstitial pneumonia.
Keywords:Adult, Aged, Algorithms, Autoantibodies, Biopsy, Connective Tissue Diseases, Female, Humans, Idiopathic Interstitial Pneumonias, Male, Middle Aged, Prognosis, Raynaud Disease, Retrospective Studies, Severity of Illness Index, Survival, Tomography, X-Ray Computed
UCL classification:UCL > School of Life and Medical Sciences > Faculty of Medical Sciences > Medicine (Division of)

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