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Treatment of systemic sclerosis
Yannick Allanore1, André Kahan
1Rheumatology A Department, Cochin Teaching Hospital, Assistance Publique-Hôpitaux de Paris, Paris 5 University, 27, rue du faubourg Saint-Jacques, 75014 Paris, France. yannick.allanore@cch.aphp.fr
Insights
Systemic sclerosis treatment shows improved survival mainly due to cardiovascular drugs like ACE inhibitors and prostacyclins, while disease-modifying agents have not impacted skin fibrosis effectively.
Area of Science:
- Rheumatology
- Immunology
- Cardiology
Background:
- Systemic sclerosis is a severe connective tissue disease with complex pathogenic processes involving vascular, immune, and fibroblast abnormalities.
- Understanding these mechanisms is crucial for evaluating treatment efficacy.
Purpose of the Study:
- To review the current treatment strategies for systemic sclerosis.
- To evaluate the effectiveness of various therapeutic agents, particularly cardiovascular drugs and disease-modifying agents.
Main Methods:
- Review of recent studies and clinical trial data on systemic sclerosis treatments.
- Analysis of treatment outcomes focusing on mortality, renal crisis, pulmonary hypertension, myocardial involvement, and skin fibrosis.
Main Results:
- Cardiovascular drugs, including Angiotensin-converting enzyme (ACE) inhibitors, prostacyclins, endothelin antagonists, and calcium antagonists, have significantly reduced excess mortality and improved specific organ involvement.
- Agents investigated for disease-modifying potential, including immunomodulatory drugs, have not demonstrated efficacy in reducing skin fibrosis in controlled trials.
- Ongoing trials are investigating immunosuppressants for their potential benefits.
Conclusions:
- Current evidence strongly supports the use of cardiovascular drugs for managing systemic sclerosis complications and improving survival.
- Further research is needed, emphasizing the development of disease activity and severity criteria to guide future treatment investigations for systemic sclerosis.
Abstract:
Systemic sclerosis is the most severe of all connective tissue diseases. The distinctive pathogenic process involves sequential or concomitant abnormalities in blood vessel function, immunity and, ultimately, fibroblast function. These specific characteristics may explain the results of treatment evaluations. The decrease in excess mortality shown in recent studies seems chiefly ascribable to the use of cardiovascular drugs. Angiotensin-converting enzyme (ACE) inhibitors are effective in resolving renal crisis, prostacyclins and endothelin antagonists improve pulmonary hypertension, and calcium antagonists and ACE inhibitors benefit patients with myocardial involvement. On the other hand, immunomodulatory drugs and other agents investigated for their disease-modifying potential failed to influence skin fibrosis in controlled trials. Trials of immunosuppressants are ongoing. Available results indicate that emphasis should be put on cardiovascular drugs. The development of criteria for disease activity and severity would facilitate future research on the treatment of systemic sclerosis.
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