Gianluca Sambataro1, Domenico Sambataro2, Sebastiano Emanuele Torrisi3, Ada Vancheri3, Michele Colaci4, Mauro Pavone3, Francesca Pignataro5, Nicoletta Del Papa5, Stefano Palmucci6, Carlo Vancheri3. 1. 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. 2. 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. 3. Regional Referral Centre for Rare Lung Diseases, A. O. U. "Policlinico-Vittorio Emanuele" Dept. of Clinical and Experimental Medicine, University of Catania, Italy. 4. Department of Clinical and Experimental Medicine, Internal Medicine Unit, Section of Rheumatology, Cannizzaro Hospital, University ofCatania, Via Messina 829, 95126, Catania, Italy. 5. Day Hospital of Rheumatology, Dept. of Rheumatology, ASST G. Pini-CTO, Milan, Italy. 6. Department of Radiology, "Policlinico-Vittorio Emanuele", University of Catania, Italy.
Abstract
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 O2 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.
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 ILDpatients 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 O2 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.
Authors: Julia Graham; Iazsmin Bauer Ventura; Chad A Newton; Cathryn Lee; Noelle Boctor; Janelle Vu Pugashetti; Claire Cutting; Elena Joerns; Habrinder Sandhu; Jonathan H Chung; Christine Kim Garcia; Michael Kadoch; Imre Noth; Ayodeji Adegunsoye; Mary E Strek; Justin M Oldham Journal: Eur Respir J Date: 2020-07-16 Impact factor: 16.671
Authors: Domenico Sambataro; Gianluca Sambataro; Francesca Pignataro; Giovanni Zanframundo; Veronica Codullo; Evelina Fagone; Emanuele Martorana; Francesco Ferro; Martina Orlandi; Nicoletta Del Papa; Lorenzo Cavagna; Lorenzo Malatino; Michele Colaci; Carlo Vancheri Journal: Diagnostics (Basel) Date: 2020-04-09