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See Latest ArticlesThe manifestations of vasculopathy in systemic sclerosis and its evidence-based therapy
International Journal of Rheumatic Diseases, 08/19/09
Strange G et al. – Review concludes that several manifestations of systemic sclerosis (SSc) including Raynaud's phenomenon, digital ulceration, renal crisis and pulmonary arterial hypertension (PAH) share similarities in their underlying vasculopathy. Multiple, available treatments can reduce the morbidity and mortality associated with these manifestations.
Methods- SSc is a connective tissue disease of unknown etiology that is characterized by inflammation, fibrosis, and vasculopathy
- Manifestations of SSc may arise in numerous organs and tissues
- This article identifies those manifestations with the most closely-associated vascular pathology, and evaluates current, available approaches for their treatment
- Several treatments have successfully been shown to target these manifestations
- However, varying levels of supporting evidence exist and no treatment has yet demonstrated improvements in overall disease course
- More robust studies support:
- Use of calcium channel blockers for Raynaud's phenomenon, and
- Treatment with bosentan or iloprost for digital ulcers
- For pts with scleroderma renal crisis, ACE inhibitors are the cornerstone of treatment
- For pts who develop PAH, use of an endothelin receptor antagonist, a phosphodiesterase inhibitor, or a prostanoid is recommended
- Early treatment, and using combinations of targeted therapies optimizes outcomes for pts with PAH
- A need exists for the standardization in management of vascular manifestations of SSc
- The creation of a treatment algorithm supported by international consensus also would be welcomed
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Nephrogenic systemic fibrosis
Current Opinion in Rheumatology, 11/04/09
Effect of Sildenafil on digital ulcers in systemic sclerosis - analysis from a single centre pilot study
Annals of Rheumatic Diseases, 11/23/09
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Clinical Rheumatology, 10/27/09
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