Abstract
Systemic sclerosis is a connective tissue disease characterized by fibrosis of the skin, internal organs, and widespread vasculopathy. Raynaud's phenomenon and digital ulcers are vascular manifestations of this disease and cause significant morbidity. Current treatments are only moderately effective in reducing the severity of Raynaud's in a portion of patients and typically do not lead to substantial benefit in terms of the healing or prevention of digital ulcers. Several studies have evaluated the efficacy of targeting the vasoconstrictor endothelin-1 for the treatment of systemic sclerosis-associated vascular disease. The purpose of this paper is to summarize the published studies and case reports evaluating the efficacy of endothelin receptor antagonists in the treatment of Raynaud's phenomenon and digital ulcers associated with systemic sclerosis.
Highlights
Systemic sclerosis (SSc) is a connective tissue disease characterized by fibrosis of the skin and internal organs and widespread vasculopathy
The purpose of this paper is to summarize the published studies evaluating endothelin receptor antagonists (ETRA) in the treatment of Raynaud’s phenomenon (RP) and/or ischemic Digital ulcers (DUs) associated with SSc
The findings from the literature reviewed here indicate that ETRAs may play a role in the treatment of RP and DU in addition to their indication for the treatment of pulmonary arterial hypertension (PAH)
Summary
Systemic sclerosis (SSc) is a connective tissue disease characterized by fibrosis of the skin and internal organs and widespread vasculopathy. Digital ulcers (DUs) are necrotic lesions that occur either at distal aspects of digits (fingers or toes) or over bony prominences and occur in up to 50% of patients with limited or diffuse cutaneous SSc [3] These lesions are exquisitely painful, heal slowly, and interfere with activities of daily living often leading to substantial functional disability. Ulcerations on the lower extremities proximal to the feet can occur in patients with SSc who likely have macrovascular disease as well Current treatments for both RP and DU consist of vasodilators including calcium channel blockers (CCBs), alpha-adrenergic inhibitors, angiotensin converting enzyme (ACE) inhibitors, angiotensin receptor blockers, and nitroglycerin analogues. These medications are moderately effective in reducing the severity of RP in a portion of SSc patients [9], but typically do not lead to substantial benefit in terms of the healing and prevention of DU
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