A broad look into the future of systemic sclerosis
Systemic sclerosis (SSc) is a systemic autoimmune disease with the key features of inflammation, vasculopathy and fibrosis. This article focussed on emerging fields based on the authors’ current work and expertise. The authors provide a hierarchical structure into the studies of the pathogenesis of...
| Main Authors: | , |
|---|---|
| Format: | Article |
| Language: | English |
| Published: |
SAGE Publishing
2022-08-01
|
| Series: | Therapeutic Advances in Musculoskeletal Disease |
| Online Access: | https://doi.org/10.1177/1759720X221109404 |
| _version_ | 1798040455535919104 |
|---|---|
| author | Gabriela Riemekasten Jörg H.W. Distler |
| author_facet | Gabriela Riemekasten Jörg H.W. Distler |
| author_sort | Gabriela Riemekasten |
| collection | DOAJ |
| description | Systemic sclerosis (SSc) is a systemic autoimmune disease with the key features of inflammation, vasculopathy and fibrosis. This article focussed on emerging fields based on the authors’ current work and expertise. The authors provide a hierarchical structure into the studies of the pathogenesis of SSc starting with the contribution of environmental factors. Regulatory autoantibodies (abs) are discussed, which are parts of the human physiology and are specifically dysregulated in SSc. Abs against the angiotensin II receptor subtype 1 (AT1R) and the endothelin receptor type A (ETAR) are discussed in more detail. Extracellular vesicles are another novel player to possess disease processes. Fibroblasts are a key effector cell in SSc. Therefore, the current review will provide an overview about their plasticity in the phenotype and function. Promising nuclear receptors as key regulators of transcriptional programmes will be introduced as well as epigenetic modifications, which are pivotal to maintain the profibrotic fibroblast phenotype independent of external stimuli. Fibroblasts from SSc patients exhibit a specific signalling and reactivate developmental pathways and stem cell maintenance such as by employing hedgehog and WNT, which promote fibroblast-to-myofibroblast transition and extracellular matrix generation. Pharmacological interventions, although for other indications, are already in clinical use to address pathologic signalling. |
| first_indexed | 2024-04-11T22:07:50Z |
| format | Article |
| id | doaj.art-40578cbd130e4bf891f9b02212db92f4 |
| institution | Directory Open Access Journal |
| issn | 1759-7218 |
| language | English |
| last_indexed | 2024-04-11T22:07:50Z |
| publishDate | 2022-08-01 |
| publisher | SAGE Publishing |
| record_format | Article |
| series | Therapeutic Advances in Musculoskeletal Disease |
| spelling | doaj.art-40578cbd130e4bf891f9b02212db92f42022-12-22T04:00:38ZengSAGE PublishingTherapeutic Advances in Musculoskeletal Disease1759-72182022-08-011410.1177/1759720X221109404A broad look into the future of systemic sclerosisGabriela RiemekastenJörg H.W. DistlerSystemic sclerosis (SSc) is a systemic autoimmune disease with the key features of inflammation, vasculopathy and fibrosis. This article focussed on emerging fields based on the authors’ current work and expertise. The authors provide a hierarchical structure into the studies of the pathogenesis of SSc starting with the contribution of environmental factors. Regulatory autoantibodies (abs) are discussed, which are parts of the human physiology and are specifically dysregulated in SSc. Abs against the angiotensin II receptor subtype 1 (AT1R) and the endothelin receptor type A (ETAR) are discussed in more detail. Extracellular vesicles are another novel player to possess disease processes. Fibroblasts are a key effector cell in SSc. Therefore, the current review will provide an overview about their plasticity in the phenotype and function. Promising nuclear receptors as key regulators of transcriptional programmes will be introduced as well as epigenetic modifications, which are pivotal to maintain the profibrotic fibroblast phenotype independent of external stimuli. Fibroblasts from SSc patients exhibit a specific signalling and reactivate developmental pathways and stem cell maintenance such as by employing hedgehog and WNT, which promote fibroblast-to-myofibroblast transition and extracellular matrix generation. Pharmacological interventions, although for other indications, are already in clinical use to address pathologic signalling.https://doi.org/10.1177/1759720X221109404 |
| spellingShingle | Gabriela Riemekasten Jörg H.W. Distler A broad look into the future of systemic sclerosis Therapeutic Advances in Musculoskeletal Disease |
| title | A broad look into the future of systemic sclerosis |
| title_full | A broad look into the future of systemic sclerosis |
| title_fullStr | A broad look into the future of systemic sclerosis |
| title_full_unstemmed | A broad look into the future of systemic sclerosis |
| title_short | A broad look into the future of systemic sclerosis |
| title_sort | broad look into the future of systemic sclerosis |
| url | https://doi.org/10.1177/1759720X221109404 |
| work_keys_str_mv | AT gabrielariemekasten abroadlookintothefutureofsystemicsclerosis AT jorghwdistler abroadlookintothefutureofsystemicsclerosis AT gabrielariemekasten broadlookintothefutureofsystemicsclerosis AT jorghwdistler broadlookintothefutureofsystemicsclerosis |