title: Nonspecific interstitial pneumonia: survival is influenced by the underlying cause.
abstract: 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.
authors.affiliation: Université Paris 13, Sorbonne Paris Cité, EA2363 "Réponses cellulaires et fonctionnelles à l'hypoxie", Bobigny, France AP-HP, Service de Pneumologie, Hôpital Avicenne, Bobigny, France hilario.nunes@avc.aphp.fr.
authors.forename: Hilario
authors.initials: H
authors.lastname: Nunes
authors.affiliation: AP-HP, Service de Pneumologie, Hôpital Avicenne, Bobigny, France.
authors.forename: Kirsten
authors.initials: K
authors.lastname: Schubel
authors.affiliation: AP-HP, Service de Radiologie, Hôpital Avicenne, Bobigny, France.
authors.forename: Diane
authors.initials: D
authors.lastname: Piver
authors.affiliation: Service de Pneumologie, Hôpital d'Amiens, Université de Picardie Jules Verne, Amiens, France.
authors.forename: Eline
authors.initials: E
authors.lastname: Magois
authors.affiliation: Université Paris Diderot, Sorbonne Paris Cité, AP-HP, Service de Pneumologie, Hôpital Saint Louis, Paris, France.
authors.forename: Séverine
authors.initials: S
authors.lastname: Feuillet
authors.affiliation: Université Paris 13, Sorbonne Paris Cité, EA2363 "Réponses cellulaires et fonctionnelles à l'hypoxie", Bobigny, France AP-HP, Service de Pneumologie, Hôpital Avicenne, Bobigny, France.
authors.forename: Yurdagul
authors.initials: Y
authors.lastname: Uzunhan
authors.affiliation: AP-HP, Service de Pneumologie, Hôpital Avicenne, Bobigny, France.
authors.forename: Zohra
authors.initials: Z
authors.lastname: Carton
authors.affiliation: Université Paris Diderot, Sorbonne Paris Cité, AP-HP, Service de Pneumologie, Hôpital Saint Louis, Paris, France.
authors.forename: Abdellatif
authors.initials: A
authors.lastname: Tazi
authors.affiliation: AP-HP, Département de Santé Publique, Hôpital Tenon, INSERM, U707, Université Paris 6 Pierre et Marie Curie, UMR-S 707, Paris, France.
authors.forename: Pierre
authors.initials: P
authors.lastname: Levy
authors.affiliation: AP-HP, Service de Radiologie, Hôpital Avicenne, Bobigny, France.
authors.forename: Pierre-Yves
authors.initials: PY
authors.lastname: Brillet
authors.affiliation: Dept of Histopathology, Royal Brompton and Harefield NHS Foundation Trust and NHLI Division, Imperial College, London, UK.
authors.forename: Andrew G
authors.initials: AG
authors.lastname: Nicholson
authors.affiliation: AP-HP, Service d'Anatomie Pathologique, Hôpital Avicenne, Bobigny, France.
authors.forename: Marianne
authors.initials: M
authors.lastname: Kambouchner
authors.affiliation: Université Paris 13, Sorbonne Paris Cité, EA2363 "Réponses cellulaires et fonctionnelles à l'hypoxie", Bobigny, France AP-HP, Service de Pneumologie, Hôpital Avicenne, Bobigny, France.
authors.forename: Dominique
authors.initials: D
authors.lastname: Valeyre
mesh_terms: D000328:Adult; D000368:Aged; D000542:Alveolitis, Extrinsic Allergic / Q000150:complications / Q000175:diagnosis / Q000276:immunology; D001323:Autoantibodies / Q000097:blood; D001706:Biopsy; D002423:Cause of Death; D003240:Connective Tissue Diseases / Q000150:complications / Q000175:diagnosis / Q000276:immunology; D003937:Diagnosis, Differential; D005260:Female; D005602:France / Q000453:epidemiology; D006801:Humans; D054988:Idiopathic Interstitial Pneumonias / Q000175:diagnosis / Q000209:etiology / Q000276:immunology / Q000401:mortality; D053208:Kaplan-Meier Estimate; D008168:Lung / Q000000981:diagnostic imaging / Q000473:pathology; D008297:Male; D008875:Middle Aged; D017063:Outcome Assessment, Health Care; D011379:Prognosis; D016016:Proportional Hazards Models; D012129:Respiratory Function Tests / Q000379:methods; D012189:Retrospective Studies; D014057:Tomography, X-Ray Computed / Q000379:methods
chemical_list: D001323:Autoantibodies