Pathophysiology of systemic sclerosis (scleroderma)
Abstract Systemic sclerosis (scleroderma) is an autoimmune‐triggered chronic fibrosing disease that affects the skin and many other organs. Its pathophysiology is complex and involves an early endothelial damage, an inflammatory infiltrate and a resulting fibrotic reaction. Based on a predisposing g...
| Main Authors: | , , |
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| Format: | Article |
| Language: | English |
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Wiley
2022-03-01
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| Series: | Kaohsiung Journal of Medical Sciences |
| Subjects: | |
| Online Access: | https://doi.org/10.1002/kjm2.12505 |
| _version_ | 1818695078025101312 |
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| author | Ann‐Helen Rosendahl Katrin Schönborn Thomas Krieg |
| author_facet | Ann‐Helen Rosendahl Katrin Schönborn Thomas Krieg |
| author_sort | Ann‐Helen Rosendahl |
| collection | DOAJ |
| description | Abstract Systemic sclerosis (scleroderma) is an autoimmune‐triggered chronic fibrosing disease that affects the skin and many other organs. Its pathophysiology is complex and involves an early endothelial damage, an inflammatory infiltrate and a resulting fibrotic reaction. Based on a predisposing genetic background, an altered balance of the acquired and the innate immune system leads to the release of many cytokines and chemokines as well as autoantibodies, which induce the activation of fibroblasts with the formation of myofibroblasts and the deposition of a stiff and rigid connective tissue. A curative treatment is still not available but remarkable progress has been made in the management of organ complications. In addition, several breakthroughs in the pathophysiology have led to new therapeutic concepts. Based on these, many new compounds have been developed during the last years, which target these different pathways and offer specific therapeutic approaches. |
| first_indexed | 2024-12-17T13:39:45Z |
| format | Article |
| id | doaj.art-c1b072c84da84f6f90fe28a3c0d4f04f |
| institution | Directory Open Access Journal |
| issn | 1607-551X 2410-8650 |
| language | English |
| last_indexed | 2024-12-17T13:39:45Z |
| publishDate | 2022-03-01 |
| publisher | Wiley |
| record_format | Article |
| series | Kaohsiung Journal of Medical Sciences |
| spelling | doaj.art-c1b072c84da84f6f90fe28a3c0d4f04f2022-12-21T21:46:19ZengWileyKaohsiung Journal of Medical Sciences1607-551X2410-86502022-03-0138318719510.1002/kjm2.12505Pathophysiology of systemic sclerosis (scleroderma)Ann‐Helen Rosendahl0Katrin Schönborn1Thomas Krieg2Translational Matrix Biology University of Cologne, Medical Faculty Cologne GermanyTranslational Matrix Biology University of Cologne, Medical Faculty Cologne GermanyTranslational Matrix Biology University of Cologne, Medical Faculty Cologne GermanyAbstract Systemic sclerosis (scleroderma) is an autoimmune‐triggered chronic fibrosing disease that affects the skin and many other organs. Its pathophysiology is complex and involves an early endothelial damage, an inflammatory infiltrate and a resulting fibrotic reaction. Based on a predisposing genetic background, an altered balance of the acquired and the innate immune system leads to the release of many cytokines and chemokines as well as autoantibodies, which induce the activation of fibroblasts with the formation of myofibroblasts and the deposition of a stiff and rigid connective tissue. A curative treatment is still not available but remarkable progress has been made in the management of organ complications. In addition, several breakthroughs in the pathophysiology have led to new therapeutic concepts. Based on these, many new compounds have been developed during the last years, which target these different pathways and offer specific therapeutic approaches.https://doi.org/10.1002/kjm2.12505extracellular matrixfibroblastfibrosisinflammation |
| spellingShingle | Ann‐Helen Rosendahl Katrin Schönborn Thomas Krieg Pathophysiology of systemic sclerosis (scleroderma) Kaohsiung Journal of Medical Sciences extracellular matrix fibroblast fibrosis inflammation |
| title | Pathophysiology of systemic sclerosis (scleroderma) |
| title_full | Pathophysiology of systemic sclerosis (scleroderma) |
| title_fullStr | Pathophysiology of systemic sclerosis (scleroderma) |
| title_full_unstemmed | Pathophysiology of systemic sclerosis (scleroderma) |
| title_short | Pathophysiology of systemic sclerosis (scleroderma) |
| title_sort | pathophysiology of systemic sclerosis scleroderma |
| topic | extracellular matrix fibroblast fibrosis inflammation |
| url | https://doi.org/10.1002/kjm2.12505 |
| work_keys_str_mv | AT annhelenrosendahl pathophysiologyofsystemicsclerosisscleroderma AT katrinschonborn pathophysiologyofsystemicsclerosisscleroderma AT thomaskrieg pathophysiologyofsystemicsclerosisscleroderma |