Treatment of systemic sclerosis associated fibrotic manifestations: Current options and future directions
Systemic sclerosis (SSc) is a complicated multisystem disease which is characterized by the highest standardized mortality ratio among all systemic rheumatic diseases with no approved therapies so far. From a pathogenetic point of view it is generally considered that autoimmunity, vasculopathy and f...
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PCO Convin S.A.
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doaj-dd3bdfe03b4348b78cc3da7d00150c692020-11-25T01:58:33ZengPCO Convin S.A.Mediterranean Journal of Rheumatology2529-198X2019-01-01301333710.31138/mjr.30.1.33MJR-30-1-33Treatment of systemic sclerosis associated fibrotic manifestations: Current options and future directionsDimitrios Daoussis0Stamatis-Nick Liossis1Department of Rheumatology, University of Patras Medical School, Patras University Hospital, Patras, GreeceDepartment of Rheumatology, University of Patras Medical School, Patras University Hospital, Patras, GreeceSystemic sclerosis (SSc) is a complicated multisystem disease which is characterized by the highest standardized mortality ratio among all systemic rheumatic diseases with no approved therapies so far. From a pathogenetic point of view it is generally considered that autoimmunity, vasculopathy and fibrosis are the main pathophysiologic processes. In this opinion article/minireview we will discuss current and future options for SSc-related fibrotic manifestations (skin thickening and lung fibrosis). Based on the results of SLS II the best treatment option for skin involvement in SSc is mycophenolate mofetil (MMF). Methotrexate (MTX) is another option which is safe and of low cost but evidence supporting its use is weak. The standard of care for SSc-ILD nowadays is MMF. Patients not responding to MMF could be treated with rituximab (RTX) or cyclophosphamide (CYC) (tocilizumab [TCZ] could be an option as well but only for patients with increased inflammatory markers). Hematopoietic stem cell transplantation (HSCT) could be considered in patients with severe/life-threatening disease who have failed conventional treatment. The most promising therapeutic approach currently been evaluated in phase 3 trials is probably the combination of MMF plus pirfenidone.https://www.mjrheum.org/assets/files/792/file164_773.pdfsystemic sclerosismycophenolate mofetilmethotrexatehematopoietic stem cell transplantationfibrotic manifestations |
collection |
DOAJ |
language |
English |
format |
Article |
sources |
DOAJ |
author |
Dimitrios Daoussis Stamatis-Nick Liossis |
spellingShingle |
Dimitrios Daoussis Stamatis-Nick Liossis Treatment of systemic sclerosis associated fibrotic manifestations: Current options and future directions Mediterranean Journal of Rheumatology systemic sclerosis mycophenolate mofetil methotrexate hematopoietic stem cell transplantation fibrotic manifestations |
author_facet |
Dimitrios Daoussis Stamatis-Nick Liossis |
author_sort |
Dimitrios Daoussis |
title |
Treatment of systemic sclerosis associated fibrotic manifestations: Current options and future directions |
title_short |
Treatment of systemic sclerosis associated fibrotic manifestations: Current options and future directions |
title_full |
Treatment of systemic sclerosis associated fibrotic manifestations: Current options and future directions |
title_fullStr |
Treatment of systemic sclerosis associated fibrotic manifestations: Current options and future directions |
title_full_unstemmed |
Treatment of systemic sclerosis associated fibrotic manifestations: Current options and future directions |
title_sort |
treatment of systemic sclerosis associated fibrotic manifestations: current options and future directions |
publisher |
PCO Convin S.A. |
series |
Mediterranean Journal of Rheumatology |
issn |
2529-198X |
publishDate |
2019-01-01 |
description |
Systemic sclerosis (SSc) is a complicated multisystem disease which is characterized by the highest standardized mortality ratio among all systemic rheumatic diseases with no approved therapies so far. From a pathogenetic point of view it is generally considered that autoimmunity, vasculopathy and fibrosis are the main pathophysiologic processes. In this opinion article/minireview we will discuss current and future options for SSc-related fibrotic manifestations (skin thickening and lung fibrosis). Based on the results of SLS II the best treatment option for skin involvement in SSc is mycophenolate mofetil (MMF). Methotrexate (MTX) is another option which is safe and of low cost but evidence supporting its use is weak. The standard of care for SSc-ILD nowadays is MMF. Patients not responding to MMF could be treated with rituximab (RTX) or cyclophosphamide (CYC) (tocilizumab [TCZ] could be an option as well but only for patients with increased inflammatory markers). Hematopoietic stem cell transplantation (HSCT) could be considered in patients with severe/life-threatening disease who have failed conventional treatment. The most promising therapeutic approach currently been evaluated in phase 3 trials is probably the combination of MMF plus pirfenidone. |
topic |
systemic sclerosis mycophenolate mofetil methotrexate hematopoietic stem cell transplantation fibrotic manifestations |
url |
https://www.mjrheum.org/assets/files/792/file164_773.pdf |
work_keys_str_mv |
AT dimitriosdaoussis treatmentofsystemicsclerosisassociatedfibroticmanifestationscurrentoptionsandfuturedirections AT stamatisnickliossis treatmentofsystemicsclerosisassociatedfibroticmanifestationscurrentoptionsandfuturedirections |
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