Pulmonary Arterial Hypertension in Systemic Sclerosis: Update on Current Treatment
نویسندگان
چکیده
PAH is defined as mean pulmonary pressure >25 mmHg at rest and >30 mmHg with exercise. The pathogenesis of PAH in systemic sclerosis involves endothelial cell injury. With endothelial cell dysfunction, there is reduced nitrous oxide and prostaglandin production, and increased endothelin-1 production. Vascular wall remodeling then follows with intimal and smooth muscle proliferation. Luminal narrowing results in slow blood flow, a hypercoagulable state with in-situ thrombosis.
منابع مشابه
Therapy of pulmonary arterial hypertension in systemic sclerosis: an update.
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