RT Journal Article
SR Electronic
T1 Significance of pulmonary involvement in systemic sclerosis (SSc)– data from the German SSc-network
JF European Respiratory Journal
JO Eur Respir J
FD European Respiratory Society
SP PA3657
DO 10.1183/13993003.congress-2018.PA3657
VO 52
IS suppl 62
A1 Michael Kreuter
A1 Francesco Bonella
A1 Nobert Blank
A1 Elise Siegert
A1 Joerg Henes
A1 Margitta Worm
A1 Cord Sunderkoetter
A1 Marc Schmalzing
A1 Alexander Kreuter
A1 Claudia Guenther
A1 Laura Susok
A1 Gabriele Zeidler
A1 Ina Koetter
A1 Ulf Mueller-Ladner
A1 Thomas Krieg
A1 Aaron Juche
A1 Tim Schmeiser
A1 Gabriele Riemekasten
A1 Elisabeth Aberer
A1 Noemi Gaebelein-Wissing
A1 Joerg H. W. Distler
A1 Miklos Sardy
A1 Christiane Pfeiffer
A1 Kathrin Kuhr
A1 Hanns-Martin Lorenz
A1 Pia Moinzadeh
A1 Nicolas Hunzelmann
YR 2018
UL http://erj.ersjournals.com/content/52/suppl_62/PA3657.abstract
AB Background: Pulmonary involvement is the leading cause of death in SSc and can manifest as interstitial lung disease (ILD), pulmonary hypertension (PAH) or a combination (ILD-PH). Aim of this analysis was to determine prevalence, clinical characteristics and outcomes of these different forms within the German SSc Network.Methods: SSc pts were analyzed for pulmonary involvement, clinical characteristics and outcome.Results: There were 3699 pts in 42 centers with a mean follow up time of 34.4±12.6 months. At baseline, ILD was frequent (29.5%), while ILD-PH and PAH had lower prevalence (7.5%, 6.1%). At the end of follow up, 32% of SSc pts had ILD, 13% ILD-PH and 7% PAH. ILD and ILD-PH were more frequent in the diffuse form (47%, 12%), while PAH did not differ between subforms. Significant differences in baseline characteristics between PAH vs. ILD-PH vs. ILD were found for age (62, 59, 54 years), sex (males: 15%, 22%, 24%) and smoking prevalence (non-smokers 49%, 63%, 57%). Mean DLCO and FVC were 56% / 93 % for PAH, 49% / 78% for ILD-PH and 56% / 81% for ILD. Significant decreases for DLCO (≥15%) and FVC (≥10%) were found in 45% / 26% in PAH, 45% / 26% for ILD-PH and 36% / 16% in ILD. All-cause mortality was 8.1% for the total cohort and differed significantly between patients without pulmonary involvement (4%), ILD (7.8%), PAH (14.2%), and ILD-PH (21%, p<0.001).Conclusions: ILD is the most prevalent pulmonary involvement in SSc, while PH-ILD is associated with the most detrimental survival. Significant differences in baseline characteristics of types of pulmonary SSc involvement may help identify patients at risk in the future.FootnotesCite this article as: European Respiratory Journal 2018 52: Suppl. 62, PA3657.This is an ERS International Congress abstract. No full-text version is available. Further material to accompany this abstract may be available at www.ers-education.org (ERS member access only).