PMID- 30961944 OWN - NLM STAT- MEDLINE DCOM- 20200501 LR - 20200501 IS - 1532-3064 (Electronic) IS - 0954-6111 (Linking) VI - 150 DP - 2019 Apr TI - Clinical, serological and radiological features of a prospective cohort of Interstitial Pneumonia with Autoimmune Features (IPAF) patients. PG - 154-160 LID - S0954-6111(19)30086-1 [pii] LID - 10.1016/j.rmed.2019.03.011 [doi] AB - BACKGROUND: The term Interstitial Pneumonia with Autoimmune Features (IPAF) describes patients with Interstitial Lung Diseases (ILDs) and clinical or serological features of autoimmune diseases insufficient to reach a specific classification of a Connective Tissue Disease (CTD). Currently, retrospective studies on IPAF patients have proven to be heterogeneous in general characteristics, outcomes and High-Resolution Computed Tomography (HRCT) pattern. This study aims to describe for the first time the clinical, serological and radiological features of a prospective cohort of IPAF patients. This cohort is then compared to a group of patients with Idiopathic Pulmonary Fibrosis (IPF). MATERIAL AND METHODS: From 626 consecutive ILD patients evaluated, 45 IPAF and a comparison cohort of 143 IPF patients were enrolled. All patients underwent clinical assessment with rheumatologic and respiratory evaluation, HRCT, Pulmonary Function Tests and Nailfold Videocapillaroscopy. RESULTS: The IPAF patients had a predominance of female gender (62.12%) with a median age of 66 years. The most common findings were: Nonspecific Interstitial Pneumonia (NSIP, 68.89%), Antinuclear Antibody positivity (17.77%) and Raynaud Phenomenon (31.11%). In comparison with IPF, IPAF patients showed younger age, better performances in Pulmonary Function Tests, less necessity of O(2) support and predominance of female sex and NSIP pattern. DISCUSSION: This is the first report of a prospective cohort of IPAF patients. IPAF patients seem to have a less severe lung disease than IPF. IPAF criteria probably need to be revisited and validated, but their capacity to recruit patients with incomplete forms or early onset of CTD could be useful for further research. CI - Copyright (c) 2019 Elsevier Ltd. All rights reserved. FAU - Sambataro, Gianluca AU - Sambataro G AD - Regional Referral Centre for Rare Lung Diseases, A. O. U. "Policlinico-Vittorio Emanuele" Dept. of Clinical and Experimental Medicine, University of Catania, Italy; Artroreuma S.R.L., Rheumatology Outpatient registered with the National Health System, Corso S. Vito 53, 95030, Mascalucia, CT, Italy. Electronic address: dottorsambataro@gmail.com. FAU - Sambataro, Domenico AU - Sambataro D AD - Artroreuma S.R.L., Rheumatology Outpatient registered with the National Health System, Corso S. Vito 53, 95030, Mascalucia, CT, Italy; Department of Clinical and Experimental Medicine, Internal Medicine Unit, Section of Rheumatology, Cannizzaro Hospital, University ofCatania, Via Messina 829, 95126, Catania, Italy. FAU - Torrisi, Sebastiano Emanuele AU - Torrisi SE AD - Regional Referral Centre for Rare Lung Diseases, A. O. U. "Policlinico-Vittorio Emanuele" Dept. of Clinical and Experimental Medicine, University of Catania, Italy. FAU - Vancheri, Ada AU - Vancheri A AD - Regional Referral Centre for Rare Lung Diseases, A. O. U. "Policlinico-Vittorio Emanuele" Dept. of Clinical and Experimental Medicine, University of Catania, Italy. FAU - Colaci, Michele AU - Colaci M AD - Department of Clinical and Experimental Medicine, Internal Medicine Unit, Section of Rheumatology, Cannizzaro Hospital, University ofCatania, Via Messina 829, 95126, Catania, Italy. FAU - Pavone, Mauro AU - Pavone M AD - Regional Referral Centre for Rare Lung Diseases, A. O. U. "Policlinico-Vittorio Emanuele" Dept. of Clinical and Experimental Medicine, University of Catania, Italy. FAU - Pignataro, Francesca AU - Pignataro F AD - Day Hospital of Rheumatology, Dept. of Rheumatology, ASST G. Pini-CTO, Milan, Italy. FAU - Del Papa, Nicoletta AU - Del Papa N AD - Day Hospital of Rheumatology, Dept. of Rheumatology, ASST G. Pini-CTO, Milan, Italy. FAU - Palmucci, Stefano AU - Palmucci S AD - Department of Radiology, "Policlinico-Vittorio Emanuele", University of Catania, Italy. FAU - Vancheri, Carlo AU - Vancheri C AD - Regional Referral Centre for Rare Lung Diseases, A. O. U. "Policlinico-Vittorio Emanuele" Dept. of Clinical and Experimental Medicine, University of Catania, Italy. LA - eng PT - Comparative Study PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20190325 PL - England TA - Respir Med JT - Respiratory medicine JID - 8908438 RN - 0 (Antibodies, Antinuclear) SB - IM MH - Aged MH - Antibodies, Antinuclear/immunology MH - Autoimmune Diseases/immunology MH - Connective Tissue Diseases/classification/epidemiology MH - Female MH - Humans MH - Idiopathic Pulmonary Fibrosis/blood/*diagnostic imaging/*immunology/physiopathology MH - Lung Diseases, Interstitial/blood/*diagnostic imaging/*immunology/physiopathology MH - Male MH - Microscopic Angioscopy/methods MH - Middle Aged MH - Prospective Studies MH - Radiography/methods MH - Raynaud Disease/epidemiology MH - Respiratory Function Tests/methods MH - Tomography, X-Ray Computed/methods OTO - NOTNLM OT - Classification criteria OT - Diagnosis OT - Idiopathic pulmonary fibrosis OT - Interstitial Pneumonia with autoimmune features OT - Interstitial lung disease OT - Undifferentiated connective tissue disease EDAT- 2019/04/10 06:00 MHDA- 2020/05/02 06:00 CRDT- 2019/04/10 06:00 PHST- 2018/11/18 00:00 [received] PHST- 2019/03/18 00:00 [revised] PHST- 2019/03/20 00:00 [accepted] PHST- 2019/04/10 06:00 [entrez] PHST- 2019/04/10 06:00 [pubmed] PHST- 2020/05/02 06:00 [medline] AID - S0954-6111(19)30086-1 [pii] AID - 10.1016/j.rmed.2019.03.011 [doi] PST - ppublish SO - Respir Med. 2019 Apr;150:154-160. doi: 10.1016/j.rmed.2019.03.011. Epub 2019 Mar 25.