PMID- 35002713 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20231105 IS - 1663-9812 (Print) IS - 1663-9812 (Electronic) IS - 1663-9812 (Linking) VI - 12 DP - 2021 TI - Autoimmune Progressive Fibrosing Interstitial Lung Disease: Predictors of Fast Decline. PG - 778649 LID - 10.3389/fphar.2021.778649 [doi] LID - 778649 AB - A subset of interstitial lung diseases (ILDs) with autoimmune traits-including connective tissue disease-associated ILD (CTD-ILD) and interstitial pneumonia with autoimmune features (IPAF)-develops progressive fibrosing (PF)-ILD. The aim of our study was to evaluate the clinical characteristics and predictors of longitudinal lung function (LF) changes in autoimmune PF-ILD patients in a real-world setting. All ILD cases with confirmed or suspected autoimmunity discussed by a multidisciplinary team (MDT) between January 2017 and June 2019 (n = 511) were reviewed, including 63 CTD-ILD and 44 IPAF patients. Detailed medical history, LF test, diffusing capacity of the lung for carbon monoxide (DLCO), 6-min walk test (6MWT), blood gas analysis (BGA), and high-resolution computer tomography (HRCT) were performed. Longitudinal follow-up for functional parameters was at least 2 years. Women were overrepresented (70.1%), and the age of the IPAF group was significantly higher as compared to the CTD-ILD group (p < 0.001). Dyspnea, crackles, and weight loss were significantly more common in the IPAF group as compared to the CTD-ILD group (84.1% vs. 58.7%, p = 0.006; 72.7% vs. 49.2%, p = 0.017; 29.6% vs. 4.8%, p = 0.001). Forced vital capacity (FVC) yearly decline was more pronounced in IPAF (53.1 +/- 0.3 vs. 16.7 +/- 0.2 ml; p = 0.294), while the majority of patients (IPAF: 68% and CTD-ILD 82%) did not deteriorate. Factors influencing progression included malignancy as a comorbidity, anti-SS-A antibodies, and post-exercise pulse increase at 6MWT. Antifibrotic therapy was administered significantly more often in IPAF as compared to CTD-ILD patients (n = 13, 29.5% vs. n = 5, 7.9%; p = 0.007), and importantly, this treatment reduced lung function decline when compared to non-treated patients. Majority of patients improved or were stable regarding lung function, and autoimmune-associated PF-ILD was more common in patients having IPAF. Functional decline predictors were anti-SS-A antibodies and marked post-exercise pulse increase at 6MWT. Antifibrotic treatments reduced progression in progressive fibrosing CTD-ILD and IPAF, emphasizing the need for guidelines including optimal treatment start and combination therapies in this special patient group. CI - Copyright (c) 2021 Nagy, Nagy, Kolonics-Farkas, Eszes, Vincze, Barczi, Tarnoki, Tarnoki, Nagy, Kiss, Maurovich-Horvat, Bohacs and Muller. FAU - Nagy, Alexandra AU - Nagy A AD - Department of Pulmonology, Semmelweis University, Budapest, Hungary. FAU - Nagy, Tamas AU - Nagy T AD - Department of Pulmonology, Semmelweis University, Budapest, Hungary. FAU - Kolonics-Farkas, Abigel Margit AU - Kolonics-Farkas AM AD - Department of Pulmonology, Semmelweis University, Budapest, Hungary. FAU - Eszes, Noemi AU - Eszes N AD - Department of Pulmonology, Semmelweis University, Budapest, Hungary. FAU - Vincze, Krisztina AU - Vincze K AD - Department of Pulmonology, Semmelweis University, Budapest, Hungary. FAU - Barczi, Eniko AU - Barczi E AD - Department of Pulmonology, Semmelweis University, Budapest, Hungary. FAU - Tarnoki, Adam Domonkos AU - Tarnoki AD AD - Medical Imaging Centre, Semmelweis University, Budapest, Hungary. FAU - Tarnoki, David Laszlo AU - Tarnoki DL AD - Medical Imaging Centre, Semmelweis University, Budapest, Hungary. FAU - Nagy, Gyorgy AU - Nagy G AD - Department of Genetics, Cell- and Immunobiology, Semmelweis University, Budapest, Hungary. AD - Department of Rheumatology and Clinical Immunology, Semmelweis University, Budapest, Hungary. FAU - Kiss, Emese AU - Kiss E AD - Department of Clinical Immunology, Adult and Pediatric Rheumatology, National Institute of Locomotor Diseases and Disabilities, Budapest, Hungary. AD - 3rd Department of Internal Medicine and Haematology, Semmelweis University, Budapest, Hungary. FAU - Maurovich-Horvat, Pal AU - Maurovich-Horvat P AD - Medical Imaging Centre, Semmelweis University, Budapest, Hungary. FAU - Bohacs, Aniko AU - Bohacs A AD - Department of Pulmonology, Semmelweis University, Budapest, Hungary. FAU - Muller, Veronika AU - Muller V AD - Department of Pulmonology, Semmelweis University, Budapest, Hungary. LA - eng PT - Journal Article DEP - 20211222 PL - Switzerland TA - Front Pharmacol JT - Frontiers in pharmacology JID - 101548923 PMC - PMC8727590 OTO - NOTNLM OT - antifibrotics OT - autoimmune disease OT - connective tissue disease (CTD) OT - interstitial pneumonia with autoimmune features (IPAF) OT - progressive fibrosing interstitial lung disease (PF-ILD) OT - treatment COIS- VM, AB, KV, NE received consultation fees from Boehringer Ingelheim during the last 5 years. The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest. EDAT- 2022/01/11 06:00 MHDA- 2022/01/11 06:01 PMCR- 2021/12/22 CRDT- 2022/01/10 09:08 PHST- 2021/09/17 00:00 [received] PHST- 2021/10/28 00:00 [accepted] PHST- 2022/01/10 09:08 [entrez] PHST- 2022/01/11 06:00 [pubmed] PHST- 2022/01/11 06:01 [medline] PHST- 2021/12/22 00:00 [pmc-release] AID - 778649 [pii] AID - 10.3389/fphar.2021.778649 [doi] PST - epublish SO - Front Pharmacol. 2021 Dec 22;12:778649. doi: 10.3389/fphar.2021.778649. eCollection 2021.