Els Vandecasteele, Karin Melsens, Amber Vanhaecke, Daniel Blockmans, Carolien Bonroy, Charlotte Carton, Ellen Deschepper, Filip De Keyser, Frédéric Houssiau, Yves Piette, Marie Vanthuyne, Koen Verbeke, Rene Westhovens, Wim A Wuyts, Ellen De Langhe, Guy Brusselle, Vanessa Smith
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.
Vandecasteele, E, Melsens, K, Vanhaecke, A, Blockmans, D, Bonroy, C, Carton, C, Deschepper, E, De Keyser, F, Houssiau, F, Piette, Y, Vanthuyne, M, Verbeke, K, Westhovens, R, Wuyts, WA, De Langhe, E, Brusselle, G & Smith, V 2021, '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', Seminars in Arthritis and Rheumatism, vol. 51, no. 5, pp. 969-976. https://doi.org/10.1016/j.semarthrit.2021.07.018
Vandecasteele, E., Melsens, K., Vanhaecke, A., Blockmans, D., Bonroy, C., Carton, C., Deschepper, E., De Keyser, F., Houssiau, F., Piette, Y., Vanthuyne, M., Verbeke, K., Westhovens, R., Wuyts, W. A., De Langhe, E., Brusselle, G., & Smith, V. (2021). 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. Seminars in Arthritis and Rheumatism, 51(5), 969-976. https://doi.org/10.1016/j.semarthrit.2021.07.018
@article{a838659485bf471aaae0d04e1f9c9f8d,
title = "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",
abstract = "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.",
keywords = "Algorithms, Humans, Incidence, Lung, Lung Diseases, Interstitial/epidemiology, Prevalence, Retrospective Studies, Scleroderma, Systemic/complications",
author = "Els Vandecasteele and Karin Melsens and Amber Vanhaecke and Daniel Blockmans and Carolien Bonroy and Charlotte Carton and Ellen Deschepper and {De Keyser}, Filip and Fr{\'e}d{\'e}ric Houssiau and Yves Piette and Marie Vanthuyne and Koen Verbeke and Rene Westhovens and Wuyts, {Wim A} and {De Langhe}, Ellen and Guy Brusselle and Vanessa Smith",
note = "Copyright {\textcopyright} 2021 The Authors. Published by Elsevier Inc. All rights reserved.",
year = "2021",
month = oct,
doi = "10.1016/j.semarthrit.2021.07.018",
language = "English",
volume = "51",
pages = "969--976",
journal = "Seminars in Arthritis and Rheumatism",
issn = "0049-0172",
publisher = "W.B. Saunders Ltd",
number = "5",
}