PMID- 36189910 OWN - NLM STAT- MEDLINE DCOM- 20230505 LR - 20240102 IS - 0392-856X (Print) IS - 0392-856X (Linking) VI - 41 IP - 5 DP - 2023 May TI - Progression and prognosis of interstitial pneumonia with autoimmune features: a longitudinal, prospective, multi-centre study. PG - 1140-1148 LID - 10.55563/clinexprheumatol/lycdca [doi] AB - OBJECTIVES: To evaluate the rate of progression towards specific autoimmune diseases (SADs) of a prospective, multi-centre cohort of patients classifiable as interstitial pneumonia with autoimmune features (IPAF). METHODS: IPAF patients were enrolled based on specific research criteria, and jointly followed by rheumatologists and pulmonologists for at least one year with clinical check-ups, serological exams including autoimmunity, capillaroscopy and high-resolution computed tomography (HRCT). Diagnostic assessment was repeated at least once a year, or earlier when deemed useful. RESULTS: We enrolled 191 IPAF patients through 95 different combinations of IPAF criteria. Of these, 24.1% progressed towards SAD, mainly in connective tissue diseases but also in microscopic polyangiitis. The IPAF patients who progressed were younger than stable IPAF patients (63+/-10 years vs. 68+/-9 years, p=0.002) and had a longer follow-up (36.9+/-18.7 vs. 29.3+/-15.7 months, p=0.007), but similar severity. No parameters were associated with overall progression, but some parameters were associated with the development of specific diagnoses: Sjogren's syndrome with positivity for SSA (p=0.007, chi2 7.4); idiopathic inflammatory myopathy with mechanic's hands (p=<0.0001, chi2 12.6), organizing pneumonia pattern (p=0.01, chi2 6.1), positivity for anti-Pm/scl (p=0.04 chi2 4.1) and anti-MDA5 (p=0.04, chi2 4.2); systemic sclerosis with palmar telangiectasias (p=<0.0001 2 18.3), positivity for anti-Scl70 (p=<0.0001 chi2 12.5) and anti-PM/Scl (p=0.001 chi2 10.1). CONCLUSIONS: IPAF patients had a rate of progression towards SAD similar to that reported in previous studies on undifferentiated connective tissue diseases, thus including some patients in which lung involvement could represent the first or even the sole clinical manifestation of a SAD. FAU - Sambataro, Gianluca AU - Sambataro G AD - Regional Referral Centre for Rare Lung Disease, A.O.U. Policlinico G.Rodolico-San Marco, University of Catania, and Artroreuma srl, Outpatient Clinic of Rheumatology, Mascalucia, Catania, Italy. dottorsambataro@gmail.com. FAU - Sambataro, Domenico AU - Sambataro D AD - Artroreuma srl, Outpatient Clinic of Rheumatology, Mascalucia, Catania, and Department of Clinical and Experimental Medicine, Internal Medicine Unit, Division of Rheumatology, Cannizzaro Hospital, University of Catania, Italy. FAU - Spicuzza, Lucia AU - Spicuzza L AD - Regional Referral Centre for Rare Lung Disease, A.O.U. Policlinico G.Rodolico-San Marco, University of Catania, Italy. FAU - Meloni, Federica AU - Meloni F AD - Department of Internal Medicine and Therapeutics, U.O.S. Transplant Center, Fondazione IRCCS Policlinico San Matteo, University of Pavia, Italy. FAU - Lorini, Giorgio AU - Lorini G AD - Department of Internal Medicine and Therapeutics, U.O.S. Transplant Center, Fondazione IRCCS Policlinico San Matteo, University of Pavia, Italy. FAU - Malatino, Lorenzo AU - Malatino L AD - Department of Clinical and Experimental Medicine, Internal Medicine Unit, Division of Rheumatology, Cannizzaro Hospital, University of Catania, Italy. FAU - Colaci, Michele AU - Colaci M AD - Department of Clinical and Experimental Medicine, Internal Medicine Unit, Division of Rheumatology, Cannizzaro Hospital, University of Catania, Italy. FAU - Sebastiani, Giandomenico AU - Sebastiani G AD - U.O.C. Reumatologia, A.O. San Camillo-Forlanini, Roma, Italy. FAU - Iuliano, Annamaria AU - Iuliano A AD - U.O.C. Reumatologia, A.O. San Camillo-Forlanini, Roma, Italy. FAU - Canofari, Claudia AU - Canofari C AD - U.O.C. Reumatologia, A.O. San Camillo-Forlanini, Roma, Italy. FAU - Luppi, Fabrizio AU - Luppi F AD - Respiratory Diseases Unit, San Gerardo Hospital, Monza University of Milano-Bicocca, Italy. FAU - Franco, Giovanni AU - Franco G AD - Respiratory Diseases Unit, San Gerardo Hospital, Monza University of Milano-Bicocca, Italy. FAU - Zanini, Umberto AU - Zanini U AD - Respiratory Diseases Unit, San Gerardo Hospital, Monza University of Milano-Bicocca, Italy. FAU - Manfredi, Andreina AU - Manfredi A AD - Rheumatology Unit, Azienda Policlinico di Modena, University of Modena and Reggio Emilia, Modena, Italy. FAU - Gozzi, Filippo AU - Gozzi F AD - Respiratory Disease Unit, Azienda Policlinico di Modena, University of Modena and Reggio Emilia, Modena, Italy. FAU - Sebastiani, Marco AU - Sebastiani M AD - Rheumatology Unit, Azienda Policlinico di Modena, University of Modena and Reggio Emilia, Modena, Italy. FAU - Palmucci, Stefano AU - Palmucci S AD - Department of Medical-Surgical Sciences and Advanced Technologies G.F. Ingrassia, Radiology I Unit, University Hospital Policlinico G. Rodolico-San Marco, Catania, Italy. FAU - Cavagna, Lorenzo AU - Cavagna L AD - Department of Internal Medicine and Therapeutics, Division of Rheumatology, Fondazione IRCCS Policlinico San Matteo, University of Pavia, Italy. FAU - Vancheri, Carlo AU - Vancheri C AD - Regional Referral Centre for Rare Lung Disease, A.O.U. Policlinico G. Rodolico-San Marco, University of Catania, Italy. LA - eng PT - Journal Article PT - Multicenter Study DEP - 20221003 PL - Italy TA - Clin Exp Rheumatol JT - Clinical and experimental rheumatology JID - 8308521 SB - IM MH - Humans MH - Prospective Studies MH - *Lung Diseases, Interstitial/diagnostic imaging/etiology MH - *Autoimmune Diseases/diagnostic imaging/complications MH - *Connective Tissue Diseases/diagnosis/diagnostic imaging MH - Prognosis EDAT- 2022/10/04 06:00 MHDA- 2023/05/05 06:42 CRDT- 2022/10/03 06:42 PHST- 2022/07/08 00:00 [received] PHST- 2022/09/19 00:00 [accepted] PHST- 2023/05/05 06:42 [medline] PHST- 2022/10/04 06:00 [pubmed] PHST- 2022/10/03 06:42 [entrez] AID - 18966 [pii] AID - 10.55563/clinexprheumatol/lycdca [doi] PST - ppublish SO - Clin Exp Rheumatol. 2023 May;41(5):1140-1148. doi: 10.55563/clinexprheumatol/lycdca. Epub 2022 Oct 3.