Cargando…

Favorable long term effects of intensified immunosuppression combined with therapeutic plasma exchange in patients with early-onset progressive systemic sclerosis-related interstitial lung disease()

OBJECTIVE: Systemic sclerosis (SSc) related mortality and morbidity remains high. Immunosuppressive therapy is considered most effective when immune activity and inflammation but not fibrosis still dominates the disease process. This study evaluated long-term intensified immunosuppression combined w...

Descripción completa

Detalles Bibliográficos
Autores principales: Potjewijd, J., Tobal, R., Silvertand, D., Gietema, H.A., Damoiseaux, J.G.M.C., van Paassen, P.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Elsevier 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9706153/
https://www.ncbi.nlm.nih.gov/pubmed/36458101
http://dx.doi.org/10.1016/j.jtauto.2022.100174
_version_ 1784840450575695872
author Potjewijd, J.
Tobal, R.
Silvertand, D.
Gietema, H.A.
Damoiseaux, J.G.M.C.
van Paassen, P.
author_facet Potjewijd, J.
Tobal, R.
Silvertand, D.
Gietema, H.A.
Damoiseaux, J.G.M.C.
van Paassen, P.
author_sort Potjewijd, J.
collection PubMed
description OBJECTIVE: Systemic sclerosis (SSc) related mortality and morbidity remains high. Immunosuppressive therapy is considered most effective when immune activity and inflammation but not fibrosis still dominates the disease process. This study evaluated long-term intensified immunosuppression combined with therapeutic plasma exchange (TPE) in early-onset progressive SSc-related interstitial lung disease (ILD). METHODS: The study cohort consisted of 161 SSc patients, with a median follow-up time of 8.9 years. The standardized mortality rate (SMR) and overall survival was calculated in patients with and without cardiopulmonary involvement. We used a standardized, pragmatic, non-randomized approach to treat 24 consecutive early progressive SSc-ILD patients with intensified immunosuppressive therapy, including plasma exchange. Outcome measurements were event-free survival (EFS), pulmonary function and safety profile. The outcome was compared with the analyzed data from the other SSc-ILD patients, who did not fulfill the inclusion criteria, and instead were treated with estimated optimal care (EOc). RESULTS: The age-adjusted SMR of all 161 SSc patients was 3.0 (CI95%; 0.32–5.68). EFS at 10 years was 49.9% in the intensified treatment group and 43.3% in the EOc group (p = 0.106). Improvement of the percentage of predicted forced vital capacity (%pFVC) and percentage of predicted diffusing capacity for carbon monoxide (%pDLco) in the intensified treatment group was +10.1% respectively +3.6%, compared to a decrease of respectively 10.8% and 7% in the EOc (p < 0.001 resp. p = 0.019). Safety analysis showed 1 death (female patient, over 75 years of age), due to pneumosepsis, in the intensified treatment group. CONCLUSION: Intensified and long-lasting immunosuppression combined with TPE is safe in early severe systemic sclerosis and is associated with improved EFS and pulmonary function as compared to the outcome in the variable but EOc group. Our findings warrant larger studies for confirmation.
format Online
Article
Text
id pubmed-9706153
institution National Center for Biotechnology Information
language English
publishDate 2022
publisher Elsevier
record_format MEDLINE/PubMed
spelling pubmed-97061532022-11-30 Favorable long term effects of intensified immunosuppression combined with therapeutic plasma exchange in patients with early-onset progressive systemic sclerosis-related interstitial lung disease() Potjewijd, J. Tobal, R. Silvertand, D. Gietema, H.A. Damoiseaux, J.G.M.C. van Paassen, P. J Transl Autoimmun VSI:Autoantibodies in disease OBJECTIVE: Systemic sclerosis (SSc) related mortality and morbidity remains high. Immunosuppressive therapy is considered most effective when immune activity and inflammation but not fibrosis still dominates the disease process. This study evaluated long-term intensified immunosuppression combined with therapeutic plasma exchange (TPE) in early-onset progressive SSc-related interstitial lung disease (ILD). METHODS: The study cohort consisted of 161 SSc patients, with a median follow-up time of 8.9 years. The standardized mortality rate (SMR) and overall survival was calculated in patients with and without cardiopulmonary involvement. We used a standardized, pragmatic, non-randomized approach to treat 24 consecutive early progressive SSc-ILD patients with intensified immunosuppressive therapy, including plasma exchange. Outcome measurements were event-free survival (EFS), pulmonary function and safety profile. The outcome was compared with the analyzed data from the other SSc-ILD patients, who did not fulfill the inclusion criteria, and instead were treated with estimated optimal care (EOc). RESULTS: The age-adjusted SMR of all 161 SSc patients was 3.0 (CI95%; 0.32–5.68). EFS at 10 years was 49.9% in the intensified treatment group and 43.3% in the EOc group (p = 0.106). Improvement of the percentage of predicted forced vital capacity (%pFVC) and percentage of predicted diffusing capacity for carbon monoxide (%pDLco) in the intensified treatment group was +10.1% respectively +3.6%, compared to a decrease of respectively 10.8% and 7% in the EOc (p < 0.001 resp. p = 0.019). Safety analysis showed 1 death (female patient, over 75 years of age), due to pneumosepsis, in the intensified treatment group. CONCLUSION: Intensified and long-lasting immunosuppression combined with TPE is safe in early severe systemic sclerosis and is associated with improved EFS and pulmonary function as compared to the outcome in the variable but EOc group. Our findings warrant larger studies for confirmation. Elsevier 2022-11-21 /pmc/articles/PMC9706153/ /pubmed/36458101 http://dx.doi.org/10.1016/j.jtauto.2022.100174 Text en © 2022 The Authors https://creativecommons.org/licenses/by/4.0/This is an open access article under the CC BY license (http://creativecommons.org/licenses/by/4.0/).
spellingShingle VSI:Autoantibodies in disease
Potjewijd, J.
Tobal, R.
Silvertand, D.
Gietema, H.A.
Damoiseaux, J.G.M.C.
van Paassen, P.
Favorable long term effects of intensified immunosuppression combined with therapeutic plasma exchange in patients with early-onset progressive systemic sclerosis-related interstitial lung disease()
title Favorable long term effects of intensified immunosuppression combined with therapeutic plasma exchange in patients with early-onset progressive systemic sclerosis-related interstitial lung disease()
title_full Favorable long term effects of intensified immunosuppression combined with therapeutic plasma exchange in patients with early-onset progressive systemic sclerosis-related interstitial lung disease()
title_fullStr Favorable long term effects of intensified immunosuppression combined with therapeutic plasma exchange in patients with early-onset progressive systemic sclerosis-related interstitial lung disease()
title_full_unstemmed Favorable long term effects of intensified immunosuppression combined with therapeutic plasma exchange in patients with early-onset progressive systemic sclerosis-related interstitial lung disease()
title_short Favorable long term effects of intensified immunosuppression combined with therapeutic plasma exchange in patients with early-onset progressive systemic sclerosis-related interstitial lung disease()
title_sort favorable long term effects of intensified immunosuppression combined with therapeutic plasma exchange in patients with early-onset progressive systemic sclerosis-related interstitial lung disease()
topic VSI:Autoantibodies in disease
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9706153/
https://www.ncbi.nlm.nih.gov/pubmed/36458101
http://dx.doi.org/10.1016/j.jtauto.2022.100174
work_keys_str_mv AT potjewijdj favorablelongtermeffectsofintensifiedimmunosuppressioncombinedwiththerapeuticplasmaexchangeinpatientswithearlyonsetprogressivesystemicsclerosisrelatedinterstitiallungdisease
AT tobalr favorablelongtermeffectsofintensifiedimmunosuppressioncombinedwiththerapeuticplasmaexchangeinpatientswithearlyonsetprogressivesystemicsclerosisrelatedinterstitiallungdisease
AT silvertandd favorablelongtermeffectsofintensifiedimmunosuppressioncombinedwiththerapeuticplasmaexchangeinpatientswithearlyonsetprogressivesystemicsclerosisrelatedinterstitiallungdisease
AT gietemaha favorablelongtermeffectsofintensifiedimmunosuppressioncombinedwiththerapeuticplasmaexchangeinpatientswithearlyonsetprogressivesystemicsclerosisrelatedinterstitiallungdisease
AT damoiseauxjgmc favorablelongtermeffectsofintensifiedimmunosuppressioncombinedwiththerapeuticplasmaexchangeinpatientswithearlyonsetprogressivesystemicsclerosisrelatedinterstitiallungdisease
AT vanpaassenp favorablelongtermeffectsofintensifiedimmunosuppressioncombinedwiththerapeuticplasmaexchangeinpatientswithearlyonsetprogressivesystemicsclerosisrelatedinterstitiallungdisease