Interstitial lung disease with and without progressive fibrosing phenotype in patients with idiopathic inflammatory myopathies: data from a large multicentric cohort
Andrea Doria,
Yannick Allanore,
Luca Iaccarino,
Elisabetta Zanatta,
Anna Ghirardello,
Jerome Avouac,
Giacomo Emmi,
Luca Quartuccio,
Mariele Gatto,
Paolo Spagnolo,
Danilo Malandrino,
Beatrice Moccaldi,
Elena Treppo,
Elisabetta Cocconcelli,
Gioele Castelli,
Chiara Giraudo,
Anna Sara Fraia,
Elena De Zorzi,
Luana Ienna,
Lorenzo Cereser,
Federico Giannelli,
Serena Bellani,
Andrea Martini,
Elisabetta Balestro
Affiliations
Andrea Doria
1Department of Medicine, University of Padua, Italy
Yannick Allanore
Service de Rhumatologie, Hôpital Cochin, APHP, Université Paris Cité Faculté de Santé, Paris, Île-de-France, France
Luca Iaccarino
Rheumatology Unit, Department of Medicine-DIMED, Padova University Hospital, Padova, Italy
Elisabetta Zanatta
Rheumatology Unit, Department of Medicine-DIMED, Padova University Hospital, Padova, Italy
Anna Ghirardello
Rheumatology Unit, Department of Medicine-DIMED, Padova University Hospital, Padova, Italy
Jerome Avouac
Service de Rhumatologie, Hôpital Cochin, APHP, Université Paris Cité Faculté de Santé, Paris, Île-de-France, France
Giacomo Emmi
Department of Experimental and Clinical Medicine, University of Firenze, and Interdisciplinary Internal Medicine Unit, Behçet Centre and Lupus Clinic, AOU Careggi Hospital of Florence, Firenze, Italy
Luca Quartuccio
Division of Rheumatology, Department of Medicine (DAME), Academic Hospital Santa Maria della Misericordia, ASUFC, University of Udine, Udine, Italy
Mariele Gatto
Rheumatology Unit, Department of Medicine-DIMED, Padova University Hospital, Padova, Italy
Paolo Spagnolo
Respiratory Disease Unit, Department of Cardiac, Thoracic, Vascular Sciences and Public Health, Padova University Hospital, Padova, Italy
Danilo Malandrino
Department of Experimental and Clinical Medicine, University of Firenze, and Interdisciplinary Internal Medicine Unit, Behçet Centre and Lupus Clinic, AOU Careggi Hospital of Florence, Firenze, Italy
Beatrice Moccaldi
Rheumatology Unit, Department of Medicine-DIMED, Padova University Hospital, Padova, Italy
Elena Treppo
Division of Rheumatology, Department of Medicine (DAME), Academic Hospital Santa Maria della Misericordia, ASUFC, University of Udine, Udine, Italy
Elisabetta Cocconcelli
Respiratory Disease Unit, Department of Cardiac, Thoracic, Vascular Sciences and Public Health, Padova University Hospital, Padova, Italy
Gioele Castelli
Respiratory Disease Unit, Department of Cardiac, Thoracic, Vascular Sciences and Public Health, Padova University Hospital, Padova, Italy
Chiara Giraudo
Unit of Advanced Clinical and Translational Imaging, Department of Medicine – DIMED, Padova University Hospital, Padova, Italy
Anna Sara Fraia
Unit of Advanced Clinical and Translational Imaging, Department of Medicine – DIMED, Padova University Hospital, Padova, Italy
Elena De Zorzi
Respiratory Disease Unit, Department of Cardiac, Thoracic, Vascular Sciences and Public Health, Padova University Hospital, Padova, Italy
Luana Ienna
Rheumatology Unit, Department of Medicine-DIMED, Padova University Hospital, Padova, Italy
Lorenzo Cereser
Institute of Radiology, Department of Medicine (DAME), Academic Hospital Santa Maria della Misericordia, ASUFC, University of Udine, Udine, Italy
Federico Giannelli
Department of Radiology, Azienda USL Toscana Centro, Mugello Hospital, Borgo San Lorenzo, Italy
Serena Bellani
Respiratory Disease Unit, Department of Cardiac, Thoracic, Vascular Sciences and Public Health, Padova University Hospital, Padova, Italy
Andrea Martini
Unit of Internal Medicine and Hepatology, Department of Medicine, University-Teaching Hospital of Padova, Padova, Italy
Elisabetta Balestro
Respiratory Disease Unit, Department of Cardiac, Thoracic, Vascular Sciences and Public Health, Padova University Hospital, Padova, Italy
Objectives Patients with connective tissue diseases can develop interstitial lung disease (ILD), leading to a progressive fibrosing ILD (PF-ILD) phenotype in some cases. We aimed to investigate the occurrence of PF-ILD in idiopathic inflammatory myopathies (IIMs), and factors potentially predicting this phenotype. Secondary aims were to assess the radiological pattern and factors associated with IIMs-ILD.Methods Patients with IIMs from our multicentric prospective cohort were retrospectively evaluated. Data were recorded at IIMs and ILD diagnosis, and during follow-up. Patients with ILD were classified according to the predominant high-resolution CT (HRCT) pattern: non-specific interstitial pneumonia (NSIP), usual interstitial pneumonia (UIP) and organising pneumonia (OP). PF-ILD was defined according to the 2022 American Thoracic Society (ATS), European Respiratory Society (ERS), Japanese Respiratory Society (JRS) and Latin American Thoracic Society (ALAT) guidelines. Univariate and multivariate analyses were performed to identify factors associated to ILD and to PF-ILD.Results Of 253 patients with IIMs, 125 (49%) had ILD: 99 (78%) at IIMs diagnosis and 26 (22%) during follow-up (21/26 within 5 years). Multivariate analysis identified anti-Jo-1, anti-MDA5, anti-Ro52, high score on manual muscle test, mechanic’s hands and Raynaud’s phenomenon as independently associated with ILD. The predominant HRCT pattern was NSIP (50% of patients), followed by UIP (28%) and OP (22%). At 1-year follow-up, PF-ILD occurred in 18% of IIMs-ILD. PF-ILD was predicted by anti-MDA5, heliotropic rash, xerostomia and xerophthalmia at univariate but not at multivariate analysis.Conclusion Patients with IIM should be carefully screened for ILD at IIMs diagnosis and yearly during follow-up. All patients with IIMs-ILD should be carefully monitored to capture ILD progression since a consistent proportion of them are expected to develop PF-ILD.
