PMID- 29693298 OWN - NLM STAT- MEDLINE DCOM- 20190909 LR - 20190909 IS - 1440-1843 (Electronic) IS - 1323-7799 (Linking) VI - 23 IP - 11 DP - 2018 Nov TI - Prognostic factors and disease behaviour of pathologically proven fibrotic non-specific interstitial pneumonia. PG - 1032-1040 LID - 10.1111/resp.13313 [doi] AB - BACKGROUND AND OBJECTIVE: Non-specific interstitial pneumonia (NSIP) has heterogeneous characteristics in terms of background, disease behaviour and prognosis. This study of fibrotic NSIP (f-NSIP) aimed to elucidate prognosis and disease behaviour from the viewpoint of clinical background and determine whether long-term change of pulmonary function could provide useful prognostic information. METHODS: We analysed the medical records of 157 consecutive patients diagnosed with f-NSIP by surgical lung biopsy. Disease behaviour was categorized into two groups depending on long-term change of pulmonary function: progressive type (relative >/=5%/year decline in the slope of forced vital capacity and/or relative >/=7.5%/year decline in the slope of %diffusing capacity of lung carbon monoxide) or stable type. Predictors of disease behaviour and prognosis were determined using logistic and Cox regression models. RESULTS: Our f-NSIP cohort included interstitial pneumonia with autoimmune features (IPAF) (36.9%), idiopathic (non-IPAF) (22.3%) and connective tissue disease-associated interstitial lung disease (40.8%). Multivariate analysis showed that idiopathic (non-IPAF) f-NSIP and progressive type disease were negative prognostic factors of mortality. Poor treatment response at 1 year was an independent predictor of progressive type disease, but was not related to survival. In terms of disease behaviour based on pulmonary function change, some patients with IPAF f-NSIP showed a progressive course. CONCLUSION: Although an IPAF diagnosis was useful for identifying good prognosis in idiopathic f-NSIP, some idiopathic f-NSIP patients with or without IPAF showed progressive disease despite therapy. The definition of progressive type disease may be useful in clinical decision-making when determining therapy for f-NSIP. CI - (c) 2018 Asian Pacific Society of Respirology. FAU - Yamakawa, Hideaki AU - Yamakawa H AUID- ORCID: 0000-0003-2480-6087 AD - Department of Respiratory Medicine, Kanagawa Cardiovascular and Respiratory Center, Yokohama, Japan. AD - Department of Respiratory Medicine, Tokyo Jikei University Hospital, Tokyo, Japan. FAU - Kitamura, Hideya AU - Kitamura H AD - Department of Respiratory Medicine, Kanagawa Cardiovascular and Respiratory Center, Yokohama, Japan. AD - Department of Respiratory Medicine, Tokyo Jikei University Hospital, Tokyo, Japan. FAU - Takemura, Tamiko AU - Takemura T AD - Department of Pathology, Japanese Red Cross Medical Center, Tokyo, Japan. FAU - Ikeda, Satoshi AU - Ikeda S AUID- ORCID: 0000-0001-5203-7911 AD - Department of Respiratory Medicine, Kanagawa Cardiovascular and Respiratory Center, Yokohama, Japan. FAU - Sekine, Akimasa AU - Sekine A AD - Department of Respiratory Medicine, Kanagawa Cardiovascular and Respiratory Center, Yokohama, Japan. FAU - Baba, Tomohisa AU - Baba T AD - Department of Respiratory Medicine, Kanagawa Cardiovascular and Respiratory Center, Yokohama, Japan. FAU - Iwasawa, Tae AU - Iwasawa T AD - Department of Radiology, Kanagawa Cardiovascular and Respiratory Center, Yokohama, Japan. FAU - Hagiwara, Eri AU - Hagiwara E AUID- ORCID: 0000-0003-0079-7462 AD - Department of Respiratory Medicine, Kanagawa Cardiovascular and Respiratory Center, Yokohama, Japan. FAU - Sato, Shinji AU - Sato S AD - Department of Rheumatology, Tokai University Hospital, Isehara, Japan. FAU - Ogura, Takashi AU - Ogura T AD - Department of Respiratory Medicine, Kanagawa Cardiovascular and Respiratory Center, Yokohama, Japan. LA - eng PT - Journal Article DEP - 20180424 PL - Australia TA - Respirology JT - Respirology (Carlton, Vic.) JID - 9616368 SB - IM MH - Aged MH - Autoimmunity MH - Biopsy/*methods MH - Cohort Studies MH - Connective Tissue Diseases/diagnosis/pathology MH - Female MH - Humans MH - Lung/pathology/physiopathology MH - *Lung Diseases, Interstitial/diagnosis/immunology/pathology MH - Male MH - Middle Aged MH - Predictive Value of Tests MH - Prognosis MH - *Pulmonary Fibrosis/diagnosis/pathology MH - Retrospective Studies MH - Tomography, X-Ray Computed/methods OTO - NOTNLM OT - connective tissue disease OT - disease behaviour OT - interstitial pneumonia with autoimmune features OT - non-specific interstitial pneumonia OT - prognosis EDAT- 2018/04/26 06:00 MHDA- 2019/09/10 06:00 CRDT- 2018/04/26 06:00 PHST- 2017/10/03 00:00 [received] PHST- 2018/02/28 00:00 [revised] PHST- 2018/03/28 00:00 [accepted] PHST- 2018/04/26 06:00 [pubmed] PHST- 2019/09/10 06:00 [medline] PHST- 2018/04/26 06:00 [entrez] AID - 10.1111/resp.13313 [doi] PST - ppublish SO - Respirology. 2018 Nov;23(11):1032-1040. doi: 10.1111/resp.13313. Epub 2018 Apr 24.