PMID- 29667101 OWN - NLM STAT- MEDLINE DCOM- 20190110 LR - 20190110 IS - 1434-9949 (Electronic) IS - 0770-3198 (Linking) VI - 37 IP - 8 DP - 2018 Aug TI - Clinical features, risk factors, and outcomes of patients with interstitial pneumonia with autoimmune features: a population-based study. PG - 2125-2132 LID - 10.1007/s10067-018-4111-5 [doi] AB - To investigate the clinical features, risk factors and outcomes of patients with interstitial pneumonia with autoimmune features (IPAF). A total of 1429 patients with idiopathic interstitial pneumonia (IIP) and undifferentiated connective tissue disease-associated interstitial lung disease (UCTD-ILD) were screened to identify patients who met IPAF criteria. Clinical, serological, and morphological features of patients with IPAF were characterized. Outcomes between patients with IPAF, UCTD-ILD, and IIP who were divided into idiopathic pulmonary fibrosis (IPF) and non-IPF groups were compared using survival as an endpoint. Patients with IPAF were much common in young female and had lower percentage of ever smoking and a significantly shorter survival than those with non-IPAF (P < 0.001). Subgroup analysis revealed that IPAF cohort survival was worse than that in non-IPF (P < 0.001), but better than that in IPF (P < 0.001). In IPAF cohort, the most common systemic symptom and serological abnormality were Raynaud's phenomenon (12.9%) and ANA >/= 1:320 (49.2%); the most frequent high-resolution computed tomography (HRCT) pattern was nonspecific interstitial pneumonia (NSIP) (61.6%). Multivariate analysis indicated that several factors including age, smoking history, organizing pneumonia (OP) pattern in HRCT, and anti-RNP positivity were independently associated with significantly worse survival. IPAF had the distinct clinical features and outcomes compared with other groups of ILD. Additional studies should be needed to explore the underlying autoimmune mechanism and to determine risk stratification in future clinical research. FAU - Dai, Jinghong AU - Dai J AD - Department of Respiratory Medicine, The Affiliated Drum Tower Hospital of Nanjing University Medical School, No. 321 Zhongshan Road, Nanjing, 210008, Jiangsu, China. FAU - Wang, Lei AU - Wang L AD - Department of Respiratory Medicine, The Affiliated Drum Tower Hospital of Nanjing University Medical School, No. 321 Zhongshan Road, Nanjing, 210008, Jiangsu, China. FAU - Yan, Xin AU - Yan X AD - Department of Respiratory Medicine, The Affiliated Drum Tower Hospital of Nanjing University Medical School, No. 321 Zhongshan Road, Nanjing, 210008, Jiangsu, China. FAU - Li, Hui AU - Li H AD - Department of Respiratory Medicine, The Affiliated Drum Tower Hospital of Nanjing University Medical School, No. 321 Zhongshan Road, Nanjing, 210008, Jiangsu, China. FAU - Zhou, Kefeng AU - Zhou K AD - Department of Radiology, The Affiliated Drum Tower Hospital of Nanjing University Medical School, Nanjing, Jiangsu, China. FAU - He, Jian AU - He J AD - Department of Radiology, The Affiliated Drum Tower Hospital of Nanjing University Medical School, Nanjing, Jiangsu, China. FAU - Meng, Fanqing AU - Meng F AD - Department of Pathology, The Affiliated Drum Tower Hospital of Nanjing University Medical School, Nanjing, Jiangsu, China. FAU - Xu, Siyi AU - Xu S AD - Key Laboratory of Environmental Medicine Engineering, Ministry of Education, School of Public Health, Southeast University, Nanjing, 210009, Jiangsu, China. FAU - Liang, Geyu AU - Liang G AD - Key Laboratory of Environmental Medicine Engineering, Ministry of Education, School of Public Health, Southeast University, Nanjing, 210009, Jiangsu, China. FAU - Cai, Hourong AU - Cai H AD - Department of Respiratory Medicine, The Affiliated Drum Tower Hospital of Nanjing University Medical School, No. 321 Zhongshan Road, Nanjing, 210008, Jiangsu, China. caihourong2013@163.com. LA - eng GR - 81570058/the National Natural Science Foundation of China/ GR - ZDRCA2016058/Jiangsu Provincial Medical Talent/ GR - BE2017604/Jiangsu Social Development Project/ PT - Journal Article DEP - 20180418 PL - Germany TA - Clin Rheumatol JT - Clinical rheumatology JID - 8211469 RN - 0 (Adrenal Cortex Hormones) RN - 0 (Immunosuppressive Agents) SB - IM MH - Adrenal Cortex Hormones/therapeutic use MH - Age Factors MH - Aged MH - *Autoimmune Diseases/blood/complications/drug therapy/pathology MH - Connective Tissue Diseases/pathology MH - Female MH - Humans MH - Idiopathic Interstitial Pneumonias/blood/complications/drug therapy/pathology MH - Immunosuppressive Agents/therapeutic use MH - *Lung Diseases, Interstitial/blood/complications/drug therapy/pathology MH - Male MH - Middle Aged MH - Retrospective Studies MH - Risk Factors MH - Sex Factors MH - Smoking/epidemiology MH - Tomography, X-Ray Computed MH - Treatment Outcome OTO - NOTNLM OT - Idiopathic interstitial pneumonia OT - Interstitial pneumonia with autoimmune features OT - Outcome OT - Risk factor OT - Undifferentiated connective tissue disease EDAT- 2018/04/19 06:00 MHDA- 2019/01/11 06:00 CRDT- 2018/04/19 06:00 PHST- 2018/01/19 00:00 [received] PHST- 2018/04/10 00:00 [accepted] PHST- 2018/03/21 00:00 [revised] PHST- 2018/04/19 06:00 [pubmed] PHST- 2019/01/11 06:00 [medline] PHST- 2018/04/19 06:00 [entrez] AID - 10.1007/s10067-018-4111-5 [pii] AID - 10.1007/s10067-018-4111-5 [doi] PST - ppublish SO - Clin Rheumatol. 2018 Aug;37(8):2125-2132. doi: 10.1007/s10067-018-4111-5. Epub 2018 Apr 18.