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Case Report: Effectiveness of secukinumab in systemic sclerosis with early skin progress after autologous hematopoietic stem cell transplantation and end-stage kidney disease

Autologous hematopoietic stem cell transplantation (aHSCT) represents an effective treatment option in patients with severe forms of systemic sclerosis (SSc) by resetting the immune system. Nevertheless, secondary autoimmune disorders and progressive disease after aHSCT might necessitate renewed imm...

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Autores principales: Strunz, Patrick-Pascal, Labinsky, Hannah, Nagler, Lea-Kristin, Portegys, Jan, Froehlich, Matthias, Gernert, Michael, Schmalzing, Marc
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Frontiers Media S.A. 2023
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10693324/
https://www.ncbi.nlm.nih.gov/pubmed/38045701
http://dx.doi.org/10.3389/fimmu.2023.1294496
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author Strunz, Patrick-Pascal
Labinsky, Hannah
Nagler, Lea-Kristin
Portegys, Jan
Froehlich, Matthias
Gernert, Michael
Schmalzing, Marc
author_facet Strunz, Patrick-Pascal
Labinsky, Hannah
Nagler, Lea-Kristin
Portegys, Jan
Froehlich, Matthias
Gernert, Michael
Schmalzing, Marc
author_sort Strunz, Patrick-Pascal
collection PubMed
description Autologous hematopoietic stem cell transplantation (aHSCT) represents an effective treatment option in patients with severe forms of systemic sclerosis (SSc) by resetting the immune system. Nevertheless, secondary autoimmune disorders and progressive disease after aHSCT might necessitate renewed immunosuppressive treatments. This is particularly challenging when organ dysfunction, i.e., end-stage kidney failure, is present. In this case report, we present the unique case of a 43-year-old female patient with rapidly progressive diffuse systemic sclerosis who underwent aHSCT despite end-stage renal failure as consequence of SSc-renal crisis. Therefore, conditioning chemotherapy was performed with melphalan instead of cyclophosphamide with no occurrence of severe adverse events during the aplastic period and thereafter. After aHSCT, early disease progression of the skin occurred and was successfully treated with secukinumab. Thereby, to the best of our knowledge, we report the first case of successful aHSCT in a SSc-patient with end-stage kidney failure and also the first successful use of an IL-17 inhibitor to treat early disease progression after aHSCT.
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spelling pubmed-106933242023-12-03 Case Report: Effectiveness of secukinumab in systemic sclerosis with early skin progress after autologous hematopoietic stem cell transplantation and end-stage kidney disease Strunz, Patrick-Pascal Labinsky, Hannah Nagler, Lea-Kristin Portegys, Jan Froehlich, Matthias Gernert, Michael Schmalzing, Marc Front Immunol Immunology Autologous hematopoietic stem cell transplantation (aHSCT) represents an effective treatment option in patients with severe forms of systemic sclerosis (SSc) by resetting the immune system. Nevertheless, secondary autoimmune disorders and progressive disease after aHSCT might necessitate renewed immunosuppressive treatments. This is particularly challenging when organ dysfunction, i.e., end-stage kidney failure, is present. In this case report, we present the unique case of a 43-year-old female patient with rapidly progressive diffuse systemic sclerosis who underwent aHSCT despite end-stage renal failure as consequence of SSc-renal crisis. Therefore, conditioning chemotherapy was performed with melphalan instead of cyclophosphamide with no occurrence of severe adverse events during the aplastic period and thereafter. After aHSCT, early disease progression of the skin occurred and was successfully treated with secukinumab. Thereby, to the best of our knowledge, we report the first case of successful aHSCT in a SSc-patient with end-stage kidney failure and also the first successful use of an IL-17 inhibitor to treat early disease progression after aHSCT. Frontiers Media S.A. 2023-11-17 /pmc/articles/PMC10693324/ /pubmed/38045701 http://dx.doi.org/10.3389/fimmu.2023.1294496 Text en Copyright © 2023 Strunz, Labinsky, Nagler, Portegys, Froehlich, Gernert and Schmalzing https://creativecommons.org/licenses/by/4.0/This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) and the copyright owner(s) are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.
spellingShingle Immunology
Strunz, Patrick-Pascal
Labinsky, Hannah
Nagler, Lea-Kristin
Portegys, Jan
Froehlich, Matthias
Gernert, Michael
Schmalzing, Marc
Case Report: Effectiveness of secukinumab in systemic sclerosis with early skin progress after autologous hematopoietic stem cell transplantation and end-stage kidney disease
title Case Report: Effectiveness of secukinumab in systemic sclerosis with early skin progress after autologous hematopoietic stem cell transplantation and end-stage kidney disease
title_full Case Report: Effectiveness of secukinumab in systemic sclerosis with early skin progress after autologous hematopoietic stem cell transplantation and end-stage kidney disease
title_fullStr Case Report: Effectiveness of secukinumab in systemic sclerosis with early skin progress after autologous hematopoietic stem cell transplantation and end-stage kidney disease
title_full_unstemmed Case Report: Effectiveness of secukinumab in systemic sclerosis with early skin progress after autologous hematopoietic stem cell transplantation and end-stage kidney disease
title_short Case Report: Effectiveness of secukinumab in systemic sclerosis with early skin progress after autologous hematopoietic stem cell transplantation and end-stage kidney disease
title_sort case report: effectiveness of secukinumab in systemic sclerosis with early skin progress after autologous hematopoietic stem cell transplantation and end-stage kidney disease
topic Immunology
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10693324/
https://www.ncbi.nlm.nih.gov/pubmed/38045701
http://dx.doi.org/10.3389/fimmu.2023.1294496
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