PMID- 37109752 OWN - NLM STAT- MEDLINE DCOM- 20230501 LR - 20231102 IS - 1648-9144 (Electronic) IS - 1010-660X (Print) IS - 1010-660X (Linking) VI - 59 IP - 4 DP - 2023 Apr 19 TI - Clinical Characterization and Predictive Factors for Progression in a Cohort of Patients with Interstitial Lung Disease and Features of Autoimmunity: The Need for a Revision of IPAF Classification Criteria. LID - 10.3390/medicina59040794 [doi] LID - 794 AB - Background and Objectives: The "interstitial pneumonia with autoimmune features" (IPAF) criteria have been criticized because of the exclusion of usual interstitial pneumonia (UIP) patients with a single clinical or serological feature. To classify these patients, the term UIPAF was proposed. This study aims to describe clinical characteristics and predictive factors for progression of a cohort of interstitial lung disease (ILD) patients with at least one feature of autoimmunity, applying criteria for IPAF, specific connective tissue diseases (CTD), and a definition of UIPAF when possible. Methods: We retrospectively evaluated data on 133 consecutive patients with ILD at onset associated with at least one feature of autoimmunity, referred by pulmonologists to rheumatologists from March 2009 to March 2020. Patients received 33 (16.5-69.5) months of follow-up. Results: Among the 101 ILD patients included, 37 were diagnosed with IPAF, 53 with ILD-onset CTD, and 11 with UIPAF. IPAF patients had a lower prevalence of UIP pattern compared to CTD-ILD and UIPAF patients (10.8% vs. 32.1% vs. 100%, p < 0.01). During the follow-up, 4 IPAF (10.8%) and 2 UIPAF (18.2%) patients evolved into CTD-ILD. IPAF patients presented features not included in IPAF criteria, such as sicca syndrome (8.1%), and were more frequently affected by systemic hypertension (p < 0.01). Over one year, ILD progression (greater extent of fibrosis on HRCT and/or decline in PFTs) was less frequent in the IPAF group compared to CTD-ILD and UIPAF (32.3% vs. 58.8% vs. 72.7, p = 0.02). A UIP pattern and an IPAF predicted a faster (OR: 3.80, p = 0.01) and a slower (OR: 0.28, p = 0.02) ILD progression, respectively. Conclusions: IPAF criteria help identify patients who might develop a CTD-ILD, even though a single clinical or serological feature is respected. Future revisions of IPAF criteria should include sicca syndrome and separate UIP-pattern into a different definition (UIPAF), given its association with a different prognosis, independently from ILD classification. FAU - Bozzao, Francesco AU - Bozzao F AD - Internal Medicine Department, Azienda ULSS 2 "Marca Trevigiana", 31100 Treviso, Italy. FAU - Tomietto, Paola AU - Tomietto P AD - Internal Medicine Department, Rheumatology Unit, Azienda Sanitaria Universitaria Giuliano Isontina (ASUGI), 34128 Trieste, Italy. FAU - Baratella, Elisa AU - Baratella E AUID- ORCID: 0000-0002-1061-9507 AD - Institute of Radiology, Department of Medical Surgical and Health Sciences, Cattinara Hospital, University of Trieste, 34128 Trieste, Italy. AD - Department of Medicine, Surgery and Health Sciences, University of Trieste, 34128 Trieste, Italy. FAU - Kodric, Metka AU - Kodric M AD - Pneumology Unit, Azienda Sanitaria Universitaria Giuliano Isontina (ASUGI), 34128 Trieste, Italy. FAU - Cifaldi, Rossella AU - Cifaldi R AD - Pneumology Unit, Azienda Sanitaria Universitaria Giuliano Isontina (ASUGI), 34128 Trieste, Italy. FAU - Della Porta, Rossana AU - Della Porta R AD - Pneumology Unit, Azienda Sanitaria Universitaria Giuliano Isontina (ASUGI), 34128 Trieste, Italy. FAU - Prearo, Ilaria AU - Prearo I AD - Vascular Medicine Unit, University Hospital LMU Munich, 81377 Munich, Germany. FAU - Pirronello, Silvia Maria Grazia AU - Pirronello SMG AD - Emergency Medicine Unit, Azienda Ospedaliera Cannizzaro, 95126 Catania, Italy. FAU - Confalonieri, Paola AU - Confalonieri P AD - Pneumology Unit, Azienda Sanitaria Universitaria Giuliano Isontina (ASUGI), 34128 Trieste, Italy. FAU - Ruaro, Barbara AU - Ruaro B AUID- ORCID: 0000-0001-8990-859X AD - Department of Medicine, Surgery and Health Sciences, University of Trieste, 34128 Trieste, Italy. AD - Pneumology Unit, Azienda Sanitaria Universitaria Giuliano Isontina (ASUGI), 34128 Trieste, Italy. FAU - Fischetti, Fabio AU - Fischetti F AD - Internal Medicine Department, Rheumatology Unit, Azienda Sanitaria Universitaria Giuliano Isontina (ASUGI), 34128 Trieste, Italy. AD - Department of Medicine, Surgery and Health Sciences, University of Trieste, 34128 Trieste, Italy. FAU - Fabris, Bruno AU - Fabris B AD - Department of Medicine, Surgery and Health Sciences, University of Trieste, 34128 Trieste, Italy. AD - Internal Medicine Department, Azienda Sanitaria Universitaria Giuliano Isontina (ASUGI), 34128 Trieste, Italy. LA - eng PT - Journal Article DEP - 20230419 PL - Switzerland TA - Medicina (Kaunas) JT - Medicina (Kaunas, Lithuania) JID - 9425208 SB - IM MH - Humans MH - Autoimmunity MH - Retrospective Studies MH - *Sjogren's Syndrome/complications MH - Tomography, X-Ray Computed MH - *Lung Diseases, Interstitial/diagnosis MH - *Idiopathic Pulmonary Fibrosis/complications MH - *Connective Tissue Diseases/complications/diagnosis MH - Lung PMC - PMC10146211 OTO - NOTNLM OT - connective tissue diseases (CTD) OT - interstitial lung disease (ILD) OT - interstitial pneumonia with autoimmune features (IPAF) OT - progressive ILD OT - "usual" interstitial pneumonia with autoimmune features (UIPAF) COIS- The authors declare no conflict of interest. EDAT- 2023/04/28 06:41 MHDA- 2023/05/01 06:42 PMCR- 2023/04/19 CRDT- 2023/04/28 01:36 PHST- 2023/01/31 00:00 [received] PHST- 2023/04/03 00:00 [revised] PHST- 2023/04/13 00:00 [accepted] PHST- 2023/05/01 06:42 [medline] PHST- 2023/04/28 06:41 [pubmed] PHST- 2023/04/28 01:36 [entrez] PHST- 2023/04/19 00:00 [pmc-release] AID - medicina59040794 [pii] AID - medicina-59-00794 [pii] AID - 10.3390/medicina59040794 [doi] PST - epublish SO - Medicina (Kaunas). 2023 Apr 19;59(4):794. doi: 10.3390/medicina59040794.