Rheumatology Medical News about Vasculopathy, Systemic sclerosis, Treatment

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The 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

Results

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