RT Journal Article
SR Electronic
T1 Nonspecific interstitial pneumonia: survival is influenced by the underlying cause
JF European Respiratory Journal
JO Eur Respir J
FD European Respiratory Society
SP ERJ-01486-2013
DO 10.1183/09031936.00148613
A1 Hilario Nunes
A1 Kirsten Schubel
A1 Diane Piver
A1 Eline Magois
A1 Séverine Feuillet
A1 Yurdagul Uzunhan
A1 Zohra Carton
A1 Abdellatif Tazi
A1 Pierre Levy
A1 Pierre-Yves Brillet
A1 Andrew G. Nicholson
A1 Marianne Kambouchner
A1 Dominique Valeyre
YR 2014
UL http://erj.ersjournals.com/content/early/2014/12/23/09031936.00148613.abstract
AB Idiopathic, nonspecific interstitial pneumonia (NSIP) is most often associated with various clinical disorders, including connective tissue diseases (CTDs) and chronic hypersensitivity pneumonitis (cHP). Emerging evidence also suggests that “idiopathic” NSIP may be the lung manifestation of undifferentiated CTD (UCTD). However, whether or not NSIP outcome is influenced by the underlying cause remains uncertain. This retrospective study included 127 biopsy-proven NSIP patients (65 women, mean±sd age 55±12 years). Survivals were estimated using a Kaplan–Meier curve and compared using the log-rank test. Multivariate analyses were based on a Cox model. 15 (11.8%) patients had cHP, 29 (22.8%) had CTD, 32 (25.2%) satisfied the Kinder criteria for UCTD and 51 (40.1%) had idiopathic NSIP. At the end of follow-up (mean±sd 64±54 months), a difference in survival was observed between aetiological groups (p=0.002). Survival was better for UCTD than for idiopathic NSIP (p=0.020) and similar to that observed for CTD. cHP survival tended to be poorer than that of idiopathic NSIP (p=0.087) and was an independent predictor of mortality (hazard ratio 2.17, 95% CI 1.05–4.47; p=0.035). NSIP outcome is influenced by its cause. cHP exhibits the highest mortality. UCTD does not differ from CTD supporting the concept of autoimmune NSIP, with a prognosis that is better than that of idiopathic NSIP. NSIP patients should be investigated for the presence of an underlying cause, which significantly impacts survival http://ow.ly/F1lO2