PMID- 36653833 OWN - NLM STAT- MEDLINE DCOM- 20230120 LR - 20230122 IS - 1465-993X (Electronic) IS - 1465-9921 (Print) IS - 1465-9921 (Linking) VI - 24 IP - 1 DP - 2023 Jan 18 TI - Functional respiratory impairment and related factors in patients with interstitial pneumonia with autoimmune features (IPAF): Multicenter study from NEREA registry. PG - 19 LID - 10.1186/s12931-023-02317-5 [doi] LID - 19 AB - BACKGROUND: The objective of the present study is to describe the characteristics of interstitial pneumonia with autoimmune features (IPAF) patients, to assess the incidence rate of functional respiratory impairment over time and to evaluate the influence of therapeutic alternatives on the prognosis of these patients. METHODS: A longitudinal observational multicenter study was performed (NEREA registry). It was carried out by a multidisciplinary team in seven Hospitals of Madrid. Patients were included from IPAF diagnosis. MAIN OUTCOME: poor prognosis as functional respiratory impairment (relative decline in FVC % defined as >/= 5% every 6 months). Covariates: therapy, sociodemographic, clinical, radiological patterns, laboratory and functional tests. STATISTICS: Survival techniques were used to estimate IR per 100 patients-semester with their 95% confidence interval [CI]. The influence of covariates in prognosis were analyzed through cox multivariate regression models (hazard ratio (HR) and [CI]). RESULTS: 79 IPAF were included, with a mean and a maximum follow-up of 3.17 and 12 years respectively. Along the study, 77.2% received treatment (52 glucocorticoids, 25 mycophenolate, 21 azathioprine, 15 rituximab and 11 antifibrotics). IR was 23.9 [19.9-28.8], and 50% of IPAF developed functional respiratory impairment after 16 months from its diagnosis. Multivariate analysis: usual interstitial pneumonia (UIP) had poorer prognosis compared to non-specific interstitial pneumonia (NSIP) (p = 0.001). In NSIP, positive ANA, increased the risk of poor prognosis. In UIP, glucocorticoids (HR: 0.53 [0.34-0.83]), age (HR: 1.04 [1.01-1.07]), and Ro-antibodies (HR: 0.36 [0.19-0.65]) influenced the prognosis. CONCLUSIONS: IPAF have functional impairment during the first years of disease. Factors predicting deterioration differ between radiographic patterns. Our real-life study suggests the potential benefit of particular therapies in IPAF. CI - (c) 2023. The Author(s). FAU - Nieto, Maria Asuncion AU - Nieto MA AD - Pneumology Department, Hospital Clinico San Carlos, Madrid, Spain. AD - Universidad Complutense, Madrid, Spain. FAU - Sanchez-Pernaute, Olga AU - Sanchez-Pernaute O AD - Rheumatology Department, Hospital Fundacion Jimenez Diaz, Madrid, Spain. FAU - Vadillo, Cristina AU - Vadillo C AD - Rheumatology Department, Hospital Clinico San Carlos, Madrid, Spain. FAU - Rodriguez-Nieto, Maria Jesus AU - Rodriguez-Nieto MJ AD - Pneumology Department, Hospital Fundacion Jimenez Diaz, Madrid, Spain. AD - Centro de Investigacion Biomedica en Red de Enfermedades Respiratorias (CIBERES), Instituto de Salud Carlos III, Madrid, Spain. FAU - Romero-Bueno, Fredeswinda AU - Romero-Bueno F AD - Rheumatology Department, Hospital Fundacion Jimenez Diaz, Madrid, Spain. FAU - Lopez-Muniz, Belen AU - Lopez-Muniz B AD - Pneumology Department, Hospital Universitario Infanta Leonor, Madrid, Spain. FAU - Cebrian, Laura AU - Cebrian L AD - Rheumatology Department, Hospital Universitario Infanta Leonor, Madrid, Spain. FAU - Rio-Ramirez, Maria Teresa AU - Rio-Ramirez MT AD - Pneumology Department, Hospital Universitario Getafe, Madrid, Spain. FAU - Laporta, Rosalia AU - Laporta R AD - Pneumology Department, Hospital Puerta de Hierro, Madrid, Spain. FAU - Bonilla, Gema AU - Bonilla G AD - Rheumatology Department, Hospital Universitario La Paz, Madrid, Spain. FAU - Cobo, Tatiana AU - Cobo T AD - Rheumatology Department, Hospital Universitario Infanta Sofia, Madrid, Spain. FAU - Leon, Leticia AU - Leon L AD - Instituto de Investigacion Sanitaria San Carlos (IdISSC), Hospital Clinico San Carlos, Calle Martin Lagos, s/n. 28040, Madrid, Spain. lleon.hcsc@salud.madrid.org. AD - Health Sciences, Universidad Camilo Jose Cela, Madrid, Spain. lleon.hcsc@salud.madrid.org. FAU - Abasolo, Lydia AU - Abasolo L AD - Instituto de Investigacion Sanitaria San Carlos (IdISSC), Hospital Clinico San Carlos, Calle Martin Lagos, s/n. 28040, Madrid, Spain. CN - NEREA Group LA - eng PT - Letter PT - Multicenter Study DEP - 20230118 PL - England TA - Respir Res JT - Respiratory research JID - 101090633 SB - IM MH - Humans MH - *Autoimmune Diseases MH - Retrospective Studies MH - *Lung Diseases, Interstitial/diagnostic imaging/epidemiology MH - *Idiopathic Pulmonary Fibrosis MH - *Idiopathic Interstitial Pneumonias/diagnosis MH - *Respiratory Insufficiency PMC - PMC9847038 OTO - NOTNLM OT - Interstitial pneumonia with autoimmune features OT - Observational study OT - Prognosis OT - Risk factors COIS- The authors declare that they have no competing interests. FIR - Abasolo, Lydia IR - Abasolo L FIR - Nieto, Maria Asuncion IR - Nieto MA FIR - Vadillo, Cristina IR - Vadillo C FIR - Lores, Irene Martin IR - Lores IM FIR - de Castro, Ana Bustos Garcia IR - de Castro ABG FIR - Romero-Bueno, Fredeswinda IR - Romero-Bueno F FIR - Rodriguez-Nieto, Maria Jesus IR - Rodriguez-Nieto MJ FIR - Pernaute, Olga Sanchez IR - Pernaute OS FIR - Palacios, Carmelo IR - Palacios C FIR - Carrera, Luis Gomez IR - Carrera LG FIR - Bonilla, Gema IR - Bonilla G FIR - Ortega, Gemma Mora IR - Ortega GM FIR - Cobo, Tatiana IR - Cobo T FIR - Lopez-Muniz, Belen IR - Lopez-Muniz B FIR - Cebrian, Laura IR - Cebrian L FIR - Godoy, Hilda IR - Godoy H FIR - Laporta, Rosalia IR - Laporta R FIR - Cubas, Irene Llorente IR - Cubas IL FIR - Valenzuela, Claudia IR - Valenzuela C FIR - de Vicuna, Rosario Garcia IR - de Vicuna RG FIR - Jauregui, Ana IR - Jauregui A FIR - Rigual, Juan IR - Rigual J FIR - Martos, Jesus Loarce IR - Martos JL FIR - Hita, Jose Luis Morell IR - Hita JLM EDAT- 2023/01/19 06:00 MHDA- 2023/01/21 06:00 PMCR- 2023/01/18 CRDT- 2023/01/18 23:38 PHST- 2022/09/22 00:00 [received] PHST- 2023/01/05 00:00 [accepted] PHST- 2023/01/18 23:38 [entrez] PHST- 2023/01/19 06:00 [pubmed] PHST- 2023/01/21 06:00 [medline] PHST- 2023/01/18 00:00 [pmc-release] AID - 10.1186/s12931-023-02317-5 [pii] AID - 2317 [pii] AID - 10.1186/s12931-023-02317-5 [doi] PST - epublish SO - Respir Res. 2023 Jan 18;24(1):19. doi: 10.1186/s12931-023-02317-5.