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Advances in pathogenesis and treatment of systemic sclerosis

Denton, CP; (2016) Advances in pathogenesis and treatment of systemic sclerosis. Clinical Medicine , 16 (1) pp. 55-60. 10.7861/clinmedicine.16-1-55. Green open access

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Abstract

Systemic sclerosis is the most severe disease within the scleroderma spectrum and is a major medical challenge with high mortality and morbidity. There have been advances in understanding of pathogenesis that reflect the interplay between immune-inflammatory processes and vasculopathy and fibrosis. It can be regarded as a disease of connective tissue repair and this leads to organ-based complications. However the aetiology and triggering events remain to be elucidated. Treatment is available for many aspects of the disease although the available therapies are not curative and some complications remain very challenging, especially non-lethal manifestations such as fatigue, calcinosis and anorectal dysfunction. Immunosuppression is now established as a beneficial approach but balancing risk and benefit is vital, especially for powerful approaches such as autologous stem cell transplantation.

Type: Article
Title: Advances in pathogenesis and treatment of systemic sclerosis
Location: England
Open access status: An open access version is available from UCL Discovery
DOI: 10.7861/clinmedicine.16-1-55
Publisher version: http://dx.doi.org/10.7861/clinmedicine.16-1-55
Language: English
Additional information: Copyright © Royal College of Physicians 2016. All rights reserved.
Keywords: Scleroderma, autoantibodies, fibrosis, pulmonary hypertension, systemic sclerosis
UCL classification: UCL
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Medical Sciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Medical Sciences > Div of Medicine
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Medical Sciences > Div of Medicine > Inflammation
URI: https://discovery.ucl.ac.uk/id/eprint/1483336
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