A randomised, open-label trial to assess the optimal treatment strategy in early diffuse cutaneous systemic sclerosis: the UPSIDE study protocol

Introduction Systemic sclerosis (SSc) is a chronic, autoimmune connective tissue disease associated with high morbidity and mortality, especially in diffuse cutaneous SSc (dcSSc). Currently, there are several treatments available in early dcSSc that aim to change the disease course, including immuno...

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Main Authors: Vanessa Smith, Ellen De Langhe, Jacob M van Laar, Alexandre E Voskuyl, Madelon C Vonk, Roger Hesselstrand, Ulrich A Walker, Dirk M Wuttge, Reinhard E Voll, Arjan A van de Loosdrecht, Julia Spierings, Joerg Henes, Anna van Rhenen, Paco MW Welsing, Anne CA Marijnissen, Nicoletta Del Papa, Daan Dierickx, Karina R Gheorghe, Tessa Kerre, Per Ljungman, Erik WAF Marijt, Miro Mayer, Marc Schmalzing, Roland Schroers, Jeska K de Vries-Bouwstra
Format: Article
Language:English
Published: BMJ Publishing Group 2021-03-01
Series:BMJ Open
Online Access:https://bmjopen.bmj.com/content/11/3/e044483.full
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author Vanessa Smith
Ellen De Langhe
Jacob M van Laar
Alexandre E Voskuyl
Madelon C Vonk
Roger Hesselstrand
Ulrich A Walker
Dirk M Wuttge
Reinhard E Voll
Arjan A van de Loosdrecht
Julia Spierings
Joerg Henes
Anna van Rhenen
Paco MW Welsing
Anne CA Marijnissen
Nicoletta Del Papa
Daan Dierickx
Karina R Gheorghe
Tessa Kerre
Per Ljungman
Erik WAF Marijt
Miro Mayer
Marc Schmalzing
Roland Schroers
Jeska K de Vries-Bouwstra
author_facet Vanessa Smith
Ellen De Langhe
Jacob M van Laar
Alexandre E Voskuyl
Madelon C Vonk
Roger Hesselstrand
Ulrich A Walker
Dirk M Wuttge
Reinhard E Voll
Arjan A van de Loosdrecht
Julia Spierings
Joerg Henes
Anna van Rhenen
Paco MW Welsing
Anne CA Marijnissen
Nicoletta Del Papa
Daan Dierickx
Karina R Gheorghe
Tessa Kerre
Per Ljungman
Erik WAF Marijt
Miro Mayer
Marc Schmalzing
Roland Schroers
Jeska K de Vries-Bouwstra
author_sort Vanessa Smith
collection DOAJ
description Introduction Systemic sclerosis (SSc) is a chronic, autoimmune connective tissue disease associated with high morbidity and mortality, especially in diffuse cutaneous SSc (dcSSc). Currently, there are several treatments available in early dcSSc that aim to change the disease course, including immunosuppressive agents and autologous haematopoietic stem cell transplantation (HSCT). HSCT has been adopted in international guidelines and is offered in current clinical care. However, optimal timing and patient selection for HSCT are still unclear. In particular, it is unclear whether HSCT should be positioned as upfront therapy or rescue treatment for patients refractory to immunosuppressive therapy. We hypothesise that upfront HSCT is superior and results in lower toxicity and lower long-term medical costs. Therefore, we propose this randomised trial aiming to determine the optimal treatment strategy for early dcSSc by comparing two strategies used in standard care: (1) upfront autologous HSCT versus (2) immunosuppressive therapy (intravenous cyclophosphamide pulse therapy followed by mycophenolate mofetil) with rescue HSCT in case of treatment failure.Methods and analysis The UPSIDE (UPfront autologous hematopoietic Stem cell transplantation vs Immunosuppressive medication in early DiffusE cutaneous systemic sclerosis) study is a multicentre, randomised, open-label, controlled trial. In total, 120 patients with early dcSSc will be randomised. The primary outcome is event-free survival at 2 years after randomisation. Secondary outcomes include serious adverse events, functional status and health-related quality of life. We will also evaluate changes in nailfold capillaroscopy pattern, pulmonary function, cardiac MR and high-resolution CT of the chest. Follow-up visits will be scheduled 3-monthly for 2 years and annually in the following 3 years.Ethics and dissemination The study was approved by the Dutch Central Committee on Research Concerning Human Subjects (NL72607.041.20). The results will be disseminated through patient associations and conventional scientific channels.Trial registration numbers NCT04464434; NL 8720.
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spelling doaj-art-4a838e7f1e5341d79e7ae951942a0f362024-11-17T18:05:08ZengBMJ Publishing GroupBMJ Open2044-60552021-03-0111310.1136/bmjopen-2020-044483A randomised, open-label trial to assess the optimal treatment strategy in early diffuse cutaneous systemic sclerosis: the UPSIDE study protocolVanessa Smith0Ellen De Langhe1Jacob M van Laar2Alexandre E Voskuyl3Madelon C Vonk4Roger Hesselstrand5Ulrich A Walker6Dirk M Wuttge7Reinhard E Voll8Arjan A van de Loosdrecht9Julia Spierings10Joerg Henes11Anna van Rhenen12Paco MW Welsing13Anne CA Marijnissen14Nicoletta Del Papa15Daan Dierickx16Karina R Gheorghe17Tessa Kerre18Per Ljungman19Erik WAF Marijt20Miro Mayer21Marc Schmalzing22Roland Schroers23Jeska K de Vries-Bouwstra244 Rheumatology and Internal Medicine, Ghent University Hospital, Ghent, Belgium1 KU Leuven, Department of Development and Regeneration, Skeletal Biology and Engineering Research Centre, Leuven, BelgiumDepartment of Rheumatology & Clinical Immunology, UMC Utrecht, Utrecht, The NetherlandsDepartment of Rheumatology and Clinical Immunology, Amsterdam UMC, Meibergdreef 9, 1105 AZ Amsterdam, The Netherlands2 Department of Rheumatology, Radboud University Medical Center, Nijmegen, The NetherlandsDepartment of Clinical Sciences Lund, Section for Rheumatology, Lund University and Skåne University Hospital, Lund, SwedenLaboratory for Experimental Rheumatology, Department of Biomedicine, University of Basel, Basel, Basel-Stadt, SwitzerlandDepartment of Clinical Sciences, Lund, Section for Rheumatology, Lund University and Skåne University Hospital, Lund, Skåne, SwedenDepartment of Rheumatology, Medical Center-University of Freiburg, Freiburg, Baden-Württemberg, GermanyDepartment of Hematology, Amsterdam UMC Location VUmc, Amsterdam, The NetherlandsRheumatology and Clinical Immunology, Universitair Medisch Centrum Utrecht, Utrecht, The NetherlandsCenter of Interdisciplinary Rheumatology, Immunology and autoimmune diseases (INDIRA), University Hospital Tübingen, Tübingen, GermanyDepartment of Hematology, Utrecht University Medical Center, Utrecht, The NetherlandsDepartment of Rheumatology & Clinical Immunology, UMC Utrecht, Utrecht, The NetherlandsDepartment of Rheumatology & Clinical Immunology, UMC Utrecht, Utrecht, The NetherlandsScleroderma Clinic, Department of Rheumatology, ASST G. Pini-CTO, Universita degli Studi di Milano, Milano, ItalyDepartment of Haematology, KU Leuven Hospital, Leuven, Flanders, BelgiumDepartment of Rheumatology, Department of Medicine, Karolinska University Hospital, Karolinska Institutet, Stockholm, SwedenDepartment of Haematology, University Hospital Ghent, Gent, Oost-Vlaanderen, BelgiumDivision of Hematology, Department of Medicine Huddinge, Karolinska Institute, Huddinge, SwedenDepartment of Haematology, Leiden University Medical Center, Leiden, Zuid-Holland, The NetherlandsDepartment of Clinical Immunology and Rheumatology, University Hospital Centre Zagreb, Zagreb, Croatia3Department of Rheumatology and Immunology, University Hospital, Würzburg, GermanyDepartment of Medicine, Haematology and Oncology, Ruhr University of Bochum, Faculty of Medicine, Bochum, Nordrhein-Westfalen, GermanyDepartment of Rheumatology, Leiden University Medical Center, Leiden, The NetherlandsIntroduction Systemic sclerosis (SSc) is a chronic, autoimmune connective tissue disease associated with high morbidity and mortality, especially in diffuse cutaneous SSc (dcSSc). Currently, there are several treatments available in early dcSSc that aim to change the disease course, including immunosuppressive agents and autologous haematopoietic stem cell transplantation (HSCT). HSCT has been adopted in international guidelines and is offered in current clinical care. However, optimal timing and patient selection for HSCT are still unclear. In particular, it is unclear whether HSCT should be positioned as upfront therapy or rescue treatment for patients refractory to immunosuppressive therapy. We hypothesise that upfront HSCT is superior and results in lower toxicity and lower long-term medical costs. Therefore, we propose this randomised trial aiming to determine the optimal treatment strategy for early dcSSc by comparing two strategies used in standard care: (1) upfront autologous HSCT versus (2) immunosuppressive therapy (intravenous cyclophosphamide pulse therapy followed by mycophenolate mofetil) with rescue HSCT in case of treatment failure.Methods and analysis The UPSIDE (UPfront autologous hematopoietic Stem cell transplantation vs Immunosuppressive medication in early DiffusE cutaneous systemic sclerosis) study is a multicentre, randomised, open-label, controlled trial. In total, 120 patients with early dcSSc will be randomised. The primary outcome is event-free survival at 2 years after randomisation. Secondary outcomes include serious adverse events, functional status and health-related quality of life. We will also evaluate changes in nailfold capillaroscopy pattern, pulmonary function, cardiac MR and high-resolution CT of the chest. Follow-up visits will be scheduled 3-monthly for 2 years and annually in the following 3 years.Ethics and dissemination The study was approved by the Dutch Central Committee on Research Concerning Human Subjects (NL72607.041.20). The results will be disseminated through patient associations and conventional scientific channels.Trial registration numbers NCT04464434; NL 8720.https://bmjopen.bmj.com/content/11/3/e044483.full
spellingShingle Vanessa Smith
Ellen De Langhe
Jacob M van Laar
Alexandre E Voskuyl
Madelon C Vonk
Roger Hesselstrand
Ulrich A Walker
Dirk M Wuttge
Reinhard E Voll
Arjan A van de Loosdrecht
Julia Spierings
Joerg Henes
Anna van Rhenen
Paco MW Welsing
Anne CA Marijnissen
Nicoletta Del Papa
Daan Dierickx
Karina R Gheorghe
Tessa Kerre
Per Ljungman
Erik WAF Marijt
Miro Mayer
Marc Schmalzing
Roland Schroers
Jeska K de Vries-Bouwstra
A randomised, open-label trial to assess the optimal treatment strategy in early diffuse cutaneous systemic sclerosis: the UPSIDE study protocol
BMJ Open
title A randomised, open-label trial to assess the optimal treatment strategy in early diffuse cutaneous systemic sclerosis: the UPSIDE study protocol
title_full A randomised, open-label trial to assess the optimal treatment strategy in early diffuse cutaneous systemic sclerosis: the UPSIDE study protocol
title_fullStr A randomised, open-label trial to assess the optimal treatment strategy in early diffuse cutaneous systemic sclerosis: the UPSIDE study protocol
title_full_unstemmed A randomised, open-label trial to assess the optimal treatment strategy in early diffuse cutaneous systemic sclerosis: the UPSIDE study protocol
title_short A randomised, open-label trial to assess the optimal treatment strategy in early diffuse cutaneous systemic sclerosis: the UPSIDE study protocol
title_sort randomised open label trial to assess the optimal treatment strategy in early diffuse cutaneous systemic sclerosis the upside study protocol
url https://bmjopen.bmj.com/content/11/3/e044483.full
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