Gemma Lepri1, Jerome Avouac2, Paolo Airò3, Francisco Anguita Santos4, Silvia Bellando-Randone5, Jelena Blagojevic5, Francisco Garcia Hernàndez6, Jose Antonio Gonzalez Nieto7, Serena Guiducci5, Suzana Jordan8, Vidya Limaye9, Britta Maurer8, Albert Selva-O'Callaghan10, Valeria Riccieri11, Oliver Distler8, Marco Matucci-Cerinic5, Yannick Allanore2. 1. Dept. of Clinical and Experimental Medicine, AOUC, University of Florence, Italy; and Paris Descartes University, Rheumatology A Dept., APHP, Cochin Hospital, Paris, France. 2. Paris Descartes University, Rheumatology A Department, APHP, Cochin Hospital, Paris, France. 3. Spedali Civili di Brescia, Service of Rheumatology and Clinical Immunology, University of Brescia, Italy. 4. Hospital Clinico San Cecilio, Granada, Spain; on behalf of GEAS-SEMI (Grupo Enfermedades Autoinmune Sistémicas)-(Sociedad Española de Medicina Interna). 5. Department of Clinical and Experimental Medicine, AOUC, University of Florence, Italy. 6. Hospital Virgen del Rocio, Department of Internal Medicine, Sevilla, Spain; on behalf of GEAS-SEMI (Grupo Enfermedades Autoinmune Sistémicas)-(Sociedad Española de Medicina Interna). 7. Hospital Can Misses, Autoimmune Disease Unit, Internal Medicine, Ibiza, Spain; on behalf of GEAS-SEMI (Grupo Enfermedades Autoinmune Sistémicas)-(Sociedad Española de Medicina Interna). 8. Department of Rheumatology, University Hospital Zurich, Switzerland. 9. Royal Adelaide Hospital, University of Adelaide, Australia. 10. Vall D'Hebron General Hospital, Autonoma Univeristy of Barcelona, Internal Medicine, Barcelona, Spain; on behalf of GEAS-SEMI (Grupo Enfermedades Autoinmune Sistémicas)-(Sociedad Española de Medicina Interna). 11. Sapienza University of Rome, Department of Internal Medicine and Medical Specialities, Rome, Italy.
Abstract
OBJECTIVES: Interstitial lung disease (ILD) is a key prognostic factor in connective tissue disorders (CTDs). The aim of our study was to assess the changes in pulmonary functional tests (PFTs) in various CTDs, including anti-synthetase syndrome (SYN), systemic sclerosis (SSc) and mixed connective tissue disorder (MCTD), following the use of rituximab therapy. METHODS: A multicentre retrospective analysis of patients with ILD secondary to SYN (n=15), MCTD (n=6) and SSc (n=23). PFTs were performed at baseline and at 1 and 2 years of follow-up. The primary outcome was the change in forced vital capacity (FVC) at 1 year. RESULTS: In the SYN population, median FVC changed from 53.0% (42.0-90.0) at baseline to 51.4% (45.6-85.0) at 1 year and 63.0 (50-88) (p=0.6) at 2 years (p=0.14). In SSc, FVC changed from 81.0% (66.0-104.0) at baseline to 89.0% (65.0-113.0) at 1 year (p=0.1) and 74.5 (50-91) at 2 years (p=0.07). In the MCTD population, FVC changed from 64.5% (63.0-68.0) at baseline to 63.0% (59.0-71.0) at 1 year (p=0.6) and 61 (59-71) after 2 years (p=0.8). DLCO showed a trend for improvement in the SYN population (p=0.06 at 1 year and 0.2 at years) while changes remain non-significant in the SSc and MCTD patients. In SYN patients, the percentage of responders at 1 year for FVC (33.3%) was greater than in SSc (9.5%) (p=0.07) and MCTD (17%) (p=0.45). RTX showed a satisfactory safety profile. CONCLUSIONS: A trend of improvement of PFTs was observed in SYN patients although not reaching significance, while SSc and MCTD patients were stabilised.
OBJECTIVES:Interstitial lung disease (ILD) is a key prognostic factor in connective tissue disorders (CTDs). The aim of our study was to assess the changes in pulmonary functional tests (PFTs) in various CTDs, including anti-synthetase syndrome (SYN), systemic sclerosis (SSc) and mixed connective tissue disorder (MCTD), following the use of rituximab therapy. METHODS: A multicentre retrospective analysis of patients with ILD secondary to SYN (n=15), MCTD (n=6) and SSc (n=23). PFTs were performed at baseline and at 1 and 2 years of follow-up. The primary outcome was the change in forced vital capacity (FVC) at 1 year. RESULTS: In the SYN population, median FVC changed from 53.0% (42.0-90.0) at baseline to 51.4% (45.6-85.0) at 1 year and 63.0 (50-88) (p=0.6) at 2 years (p=0.14). In SSc, FVC changed from 81.0% (66.0-104.0) at baseline to 89.0% (65.0-113.0) at 1 year (p=0.1) and 74.5 (50-91) at 2 years (p=0.07). In the MCTD population, FVC changed from 64.5% (63.0-68.0) at baseline to 63.0% (59.0-71.0) at 1 year (p=0.6) and 61 (59-71) after 2 years (p=0.8). DLCO showed a trend for improvement in the SYN population (p=0.06 at 1 year and 0.2 at years) while changes remain non-significant in the SSc and MCTDpatients. In SYNpatients, the percentage of responders at 1 year for FVC (33.3%) was greater than in SSc (9.5%) (p=0.07) and MCTD (17%) (p=0.45). RTX showed a satisfactory safety profile. CONCLUSIONS: A trend of improvement of PFTs was observed in SYNpatients although not reaching significance, while SSc and MCTDpatients were stabilised.
Authors: Marco A Alba; Luis Felipe Flores-Suárez; Ashley G Henderson; Hong Xiao; Peiqi Hu; Patrick H Nachman; Ronald J Falk; J Charles Jennette Journal: Autoimmun Rev Date: 2017-05-04 Impact factor: 9.754
Authors: Alejandro Robles-Perez; Jordi Dorca; Ivan Castellví; Joan Miquel Nolla; Maria Molina-Molina; Javier Narváez Journal: Rheumatol Int Date: 2020-03-10 Impact factor: 2.631
Authors: Tracy J Doyle; Namrata Dhillon; Rachna Madan; Fernanda Cabral; Elaine A Fletcher; Diane C Koontz; Rohit Aggarwal; Juan C Osorio; Ivan O Rosas; Chester V Oddis; Paul F Dellaripa Journal: J Rheumatol Date: 2018-04-01 Impact factor: 4.666