• Gianluca Sambataro, Domenico Sambataro, Sebastiano Emanuele Torrisi, Ada Vancheri, Michele Colaci, Mauro Pavone, Francesca Pignataro, Nicoletta Del Papa, Stefano Palmucci, and Carlo Vancheri.
• 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.
• Respir Med. 2019 Apr 1; 150: 154-160.

BackgroundThe 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 MethodsFrom 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.ResultsThe 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 O2 support and predominance of female sex and NSIP pattern.DiscussionThis 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.Copyright © 2019 Elsevier Ltd. All rights reserved.

39 keywords...

hide…