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Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease
Published on: June 16, 2020
Systemic sclerosis: an update in 2008
Yannick Allanore1, Jérôme Avouac, André Kahan
1Paris Descartes University, Medical Faculty, Department of Rheumatology A, Cochin Hospital, AP-HP and INSERM U781, Necker Hospital, Paris, France. yannick.allanore@cch.aphp.fr
Abstract:
The pathogenic process of systemic sclerosis targets the skin and internal organs, and involves sequential or concomitant abnormalities in blood vessel function, in immunity and inflammation, and in subsequent fibroblast activation. These characteristics are disease-specific and may partly explain the unresolved therapeutic strategies. These latter must take into account not only the broad biological abnormalities but also the complexities of the disturbances throughout the duration of the disease. However, recent epidemiological data have suggested a decrease in excess mortality, which may be mostly due to the use of cardiovascular drugs. Indeed, the prognoses of the kidney, heart and pulmonary vascular involvements have been improved. Nevertheless and despite several trials, pulmonary fibrosis remains a challenge with no clear efficacy of the regimen of immunosuppressors that have been investigated. This article deals with current and possible future therapeutic options.
Insights
Systemic sclerosis treatment is complex, affecting blood vessels, immunity, and fibroblasts. Cardiovascular drugs improve outcomes, but pulmonary fibrosis remains a challenge.
Area of Science:
- Rheumatology and Immunology
- Dermatology
- Cardiovascular Medicine
Background:
- Systemic sclerosis pathogenesis involves vascular, immune, and fibroblast abnormalities.
- Disease complexity complicates therapeutic strategies.
- Recent data show decreased mortality, potentially linked to cardiovascular drug use.
Purpose of the Study:
- To review current and future therapeutic options for systemic sclerosis.
- To discuss the impact of cardiovascular drugs on systemic sclerosis prognosis.
- To highlight challenges in treating pulmonary fibrosis in systemic sclerosis.
Main Methods:
- Review of epidemiological data on systemic sclerosis mortality.
- Analysis of treatment efficacy for various systemic sclerosis involvements.
- Discussion of current and emerging therapeutic strategies.
Main Results:
- Cardiovascular drugs have improved prognosis for kidney, heart, and pulmonary vascular involvements.
- Pulmonary fibrosis remains a therapeutic challenge despite immunosuppressor trials.
- Systemic sclerosis management requires addressing broad biological abnormalities and disease duration.
Conclusions:
- Improved cardiovascular care has positively impacted systemic sclerosis mortality.
- Pulmonary fibrosis treatment in systemic sclerosis requires novel approaches.
- Future systemic sclerosis therapies must integrate multifaceted disease characteristics.
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