Marco Sebastiani1, Giulia Cassone2, Lisa De Pasquale1, Stefania Cerri3, Giovanni Della Casa4, Caterina Vacchi1, Fabrizio Luppi3, Carlo Salvarani5, Andreina Manfredi1. 1. Chair and Rheumatology Unit, University of Modena and Reggio Emilia, Azienda Ospedaliero-Universitaria Policlinico di Modena, Modena, Italy. 2. Chair and Rheumatology Unit, University of Modena and Reggio Emilia, Azienda Ospedaliero-Universitaria Policlinico di Modena, Modena, Italy; Clinical and Experimental Medicine PhD Program, University of Modena and Reggio Emilia, Modena, Italy; Rheumatology Unit, Azienda USL-IRCCS di Reggio Emilia, Reggio Emilia, Italy. 3. Respiratory Disease Unit, University of Modena and Reggio Emilia, Azienda Ospedaliero-Universitaria Policlinico di Modena, Modena, Italy. 4. Radiology Unit, University of Modena and Reggio Emilia, Azienda Ospedaliero-Universitaria Policlinico di Modena, Modena, Italy. 5. Chair and Rheumatology Unit, University of Modena and Reggio Emilia, Azienda Ospedaliero-Universitaria Policlinico di Modena, Modena, Italy; Rheumatology Unit, Azienda USL-IRCCS di Reggio Emilia, Reggio Emilia, Italy. Electronic address: carlo.salvarani@unimore.it.
Abstract
BACKGROUND AND OBJECTIVE: Recently the term "interstitial pneumonia with autoimmune features" (IPAF) has been proposed to identify patients with interstitial lung disease and autoimmune characteristics, not fulfilling the criteria for specific connective tissue diseases (CTD). Only few data are available about the clinical and serological features of IPAF patients, their survival and the possible evolution in a CTD. The aims of the study were to investigate the demographic and clinico-serologic features of patients with IPAF, their relationship to survival, and the possible evolution in a definite CTD. PATIENTS AND METHODS: Fifty-two patients were consecutively enrolled and prospectively followed for 45 ± 31.6 months. Data about disease onset, serological, clinical and therapeutic features, pulmonary function tests and high-resolution computed tomography were periodically repeated. The survival of patients with IPAF was compared with that of 104 patients with idiopathic pulmonary fibrosis (IPF). RESULTS: The clinical domain for IPAF was satisfied in 44 patients, serological domain in 49 and the morphological domain in 29 patients. During the follow-up, a definite CTD was diagnosed in 7 patients, in particular Sjogren's syndrome in 4 patients, rheumatoid arthritis in 2, and polymyositis in the last. The estimated 5-year survival of IPAF patients 69.5 ± 7.8%, significantly higher than survival observed in IPF patients, and the baseline value of FVC and DLCO were the only factors associated to death. CONCLUSIONS: IPAF seems to a distinct entity, with a low tendency to evolve in a definite CTD. Nevertheless, further studies are needed to better define the clinical evolution and the outcome of IPAF.
BACKGROUND AND OBJECTIVE: Recently the term "interstitial pneumonia with autoimmune features" (IPAF) has been proposed to identify patients with interstitial lung disease and autoimmune characteristics, not fulfilling the criteria for specific connective tissue diseases (CTD). Only few data are available about the clinical and serological features of IPAF patients, their survival and the possible evolution in a CTD. The aims of the study were to investigate the demographic and clinico-serologic features of patients with IPAF, their relationship to survival, and the possible evolution in a definite CTD. PATIENTS AND METHODS: Fifty-two patients were consecutively enrolled and prospectively followed for 45 ± 31.6 months. Data about disease onset, serological, clinical and therapeutic features, pulmonary function tests and high-resolution computed tomography were periodically repeated. The survival of patients with IPAF was compared with that of 104 patients with idiopathic pulmonary fibrosis (IPF). RESULTS: The clinical domain for IPAF was satisfied in 44 patients, serological domain in 49 and the morphological domain in 29 patients. During the follow-up, a definite CTD was diagnosed in 7 patients, in particular Sjogren's syndrome in 4 patients, rheumatoid arthritis in 2, and polymyositis in the last. The estimated 5-year survival of IPAF patients 69.5 ± 7.8%, significantly higher than survival observed in IPFpatients, and the baseline value of FVC and DLCO were the only factors associated to death. CONCLUSIONS: IPAF seems to a distinct entity, with a low tendency to evolve in a definite CTD. Nevertheless, further studies are needed to better define the clinical evolution and the outcome of IPAF.
Authors: Elena K Joerns; Traci N Adams; Jeffrey A Sparks; Chad A Newton; Bonnie Bermas; David Karp; Una E Makris Journal: Curr Rheumatol Rep Date: 2022-06-01 Impact factor: 4.686
Authors: Marco Sebastiani; Paola Faverio; Andreina Manfredi; Giulia Cassone; Caterina Vacchi; Anna Stainer; Maria Rosa Pozzi; Carlo Salvarani; Alberto Pesci; Fabrizio Luppi Journal: Biomedicines Date: 2020-12-26
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