Alexandra Forestier1, Noémie Le Gouellec1, Hélène Béhal2, Gerdien Kramer3, Thierry Perez4, Vincent Sobanski1, Sandrine Morell Dubois1, Marc Lambert1, Pierre-Yves Hatron1, Eric Hachulla1, Alain Duhamel2, Régis Matran4, David Launay5, Martine Rémy-Jardin3. 1. University Lille, U995, Lille Inflammation Research International Center (LIRIC), F-59000 Lille, France; Inserm, U995, F-59000 Lille, France; Department of Internal Medicine and Clinical Immunology, CHU Lille, Hôpital Claude Huriez, 59037 Lille, France; Referral Centre for Rare Systemic Auto-immune Diseases North and North-West of France (CERAINO), F-59000 Lille, France. 2. University Lille, CHU Lille, EA 2694 - Santé Publique: Épidémiologie et Qualité des Soins, Unité de Biostatistiques, F-59000 Lille, France. 3. Département d'Imagerie Thoracique, CHU Lille, F-59000 Lille, France. 4. Service d'Explorations Fonctionnelles Respiratoires, INSERM U1019 - CNRS UMR 8204, CHU Lille, F-59000 Lille, France. 5. University Lille, U995, Lille Inflammation Research International Center (LIRIC), F-59000 Lille, France; Inserm, U995, F-59000 Lille, France; Department of Internal Medicine and Clinical Immunology, CHU Lille, Hôpital Claude Huriez, 59037 Lille, France; Referral Centre for Rare Systemic Auto-immune Diseases North and North-West of France (CERAINO), F-59000 Lille, France. Electronic address: david.launay@univ-lille.fr.
Abstract
OBJECTIVE: The aims of our study were to describe the evolution of interstitial lung disease (ILD) extent on HRCT scan in systemic sclerosis (SSc), to identify baseline prognostic factors associated with ILD evolution and to assess whether the evolution of pulmonary function tests (PFTs) correlated with this evolution. METHODS: 58 SSc with ILD (SSc-ILD) patients were included. All HRCT scans and PFTs available were collected. We modelized PFTs and HRCT scans evolution using linear mixed model with random effect. RESULTS: Patients underwent a median number of 3 HRCT scans (total n = 203) and 5 PFTs (total n = 329), during a mean follow-up of 5.3 ± 4.9 years. Mean SSc duration was 2.5 ± 3.1 years at the diagnosis of ILD. Mean baseline ILD extent was 32.3 ± 28.7%. We found a significant mean progression of ILD extent on serial HRCT scans of 0.92 ± 0.36% per year (p = 0.018). Male sex, diffuse cutaneous SSc (dcSSc), presence of anti-topoisomerase 1 antibodies, a higher DLCO, limited ILD and a low coarseness score at baseline in bivariate analysis, and presence of antitopoisomerase 1 antibodies and a coarseness score of 0 in multivariate analysis, were associated with faster progression of ILD extent over time There was a significant correlation between the progression of ILD extent and the decline of DLCO but only a trend for FVC. ILD extent at baseline and during follow-up was associated with survival. CONCLUSION: Male sex, dcSSc, anti-topoisomerase 1 antibodies and a less severe ILD at baseline were associated with a faster progression of ILD over time. Evolution of DLCO significantly correlated with change in ILD extent on HRCT scan. Our study helps defining the profile of patients at risk of experiencing a progression of ILD on HRCT scans.
OBJECTIVE: The aims of our study were to describe the evolution of interstitial lung disease (ILD) extent on HRCT scan in systemic sclerosis (SSc), to identify baseline prognostic factors associated with ILD evolution and to assess whether the evolution of pulmonary function tests (PFTs) correlated with this evolution. METHODS: 58 SSc with ILD (SSc-ILD) patients were included. All HRCT scans and PFTs available were collected. We modelized PFTs and HRCT scans evolution using linear mixed model with random effect. RESULTS:Patients underwent a median number of 3 HRCT scans (total n = 203) and 5 PFTs (total n = 329), during a mean follow-up of 5.3 ± 4.9 years. Mean SSc duration was 2.5 ± 3.1 years at the diagnosis of ILD. Mean baseline ILD extent was 32.3 ± 28.7%. We found a significant mean progression of ILD extent on serial HRCT scans of 0.92 ± 0.36% per year (p = 0.018). Male sex, diffuse cutaneous SSc (dcSSc), presence of anti-topoisomerase 1 antibodies, a higher DLCO, limited ILD and a low coarseness score at baseline in bivariate analysis, and presence of antitopoisomerase 1 antibodies and a coarseness score of 0 in multivariate analysis, were associated with faster progression of ILD extent over time There was a significant correlation between the progression of ILD extent and the decline of DLCO but only a trend for FVC. ILD extent at baseline and during follow-up was associated with survival. CONCLUSION: Male sex, dcSSc, anti-topoisomerase 1 antibodies and a less severe ILD at baseline were associated with a faster progression of ILD over time. Evolution of DLCO significantly correlated with change in ILD extent on HRCT scan. Our study helps defining the profile of patients at risk of experiencing a progression of ILD on HRCT scans.
Authors: Nicholas Landini; Martina Orlandi; Cosimo Bruni; Edoardo Carlesi; Cosimo Nardi; Linda Calistri; Giovanni Morana; Sara Tomassetti; Stefano Colagrande; Marco Matucci-Cerinic Journal: Front Med (Lausanne) Date: 2022-01-27