Incidence, prevalence and long-term progression of Goh algorithm rated interstitial lung disease in systemic sclerosis in two independent cohorts in flanders : a retrospective cohort study

Objectives: The epidemiology of interstitial lung disease (ILD) in systemic sclerosis (SSc) in Belgium is unknown. In literature, its prevalence varies between 19% and 52% in limited/diffuse cutaneous SSc (LcSSc/ DcSSc). However, its prevalence in "early" SSc (pre-clinically overt SSc without [yet] skin involvement), nor its incidence rate in SSc (LcSSc/DcSSc/"early" SSc) has ever been described. Against this background, we aimed to determine the prevalence/incidence (rate) and progression of ILD in SSc. Methods: 12-year follow-up data of consecutive SSc patients, included in two Flemish cohor... Mehr ...

Verfasser: Vandecasteele, Els
Melsens, Karin
Vanhaecke, Amber
Blockmans, Daniel
Bonroy, Carolien
Carton, Charlotte
Deschepper, Ellen
De Keyser, Filip
Houssiau, Frederic
Piette, Yves
Vanthuyne, Marie
Verbeke, Koen
Westhovens, Rene
Wuyts, Wim A.
De Langhe, Ellen
Brusselle, Guy
Smith, Vanessa
Dokumenttyp: journalarticle
Erscheinungsdatum: 2021
Schlagwörter: Medicine and Health Sciences / 6-MINUTE WALK TEST / CLASSIFICATION / CRITERIA / INVOLVEMENT / MORTALITY / SUBSETS / Systemic sclerosis / Early systemic sclerosis / Interstitial lung disease / Epidemiology / Prevalence / Incidence and progression
Sprache: Englisch
Permalink: https://search.fid-benelux.de/Record/base-29058226
Datenquelle: BASE; Originalkatalog
Powered By: BASE
Link(s) : https://biblio.ugent.be/publication/8742220

Objectives: The epidemiology of interstitial lung disease (ILD) in systemic sclerosis (SSc) in Belgium is unknown. In literature, its prevalence varies between 19% and 52% in limited/diffuse cutaneous SSc (LcSSc/ DcSSc). However, its prevalence in "early" SSc (pre-clinically overt SSc without [yet] skin involvement), nor its incidence rate in SSc (LcSSc/DcSSc/"early" SSc) has ever been described. Against this background, we aimed to determine the prevalence/incidence (rate) and progression of ILD in SSc. Methods: 12-year follow-up data of consecutive SSc patients, included in two Flemish cohorts (University Hospitals Ghent and Leuven), were retrospectively analysed. ILD was classified according to the simplified Goh algorithm. Progression of ILD was defined as a relative decline of FVC >10%, a combined relative decline of FVC 5-10% and DLCO >15%, or as an increase in HRCT extent. Results: 722 patients (60% LcSSc/ 20% DcSSc/ 20% "early" SSc, median (IQR) follow-up 39 [12-80] months) had baseline HRCT. 243 were rated to have ILD at baseline and 39 during follow-up (prevalence of 34%/ incidence rate of 20.3/1000PY, 95%CI:14.5-27.8). Amongst those with baseline ILD, 60% had lung functional progression at five years of follow-up. In the "early" SSc subgroup, eight patients were rated to have ILD at baseline and three during follow-up (prevalence of 6%/ incidence rate of 5.8/1000 PY, 95%CI:1.2-17.0). Conclusion: Both LcSSc and DcSSc patients should be monitored for ILD evolution. The low prevalence and incidence of ILD in the "early" SSc subgroup may instruct future decisions on the construction of uniform patient follow-up pathways in "early" SSc. (c) 2021 The Authors. Published by Elsevier Inc. This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/)