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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
Joint Bone Spine
|February 24, 2006
Summary
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.