期刊
INTEGRATED BLOOD PRESSURE CONTROL
卷 13, 期 -, 页码 15-29出版社
DOVE MEDICAL PRESS LTD
DOI: 10.2147/IBPC.S232038
关键词
systemic sclerosis; scleroderma; pulmonary arterial hypertension; pulmonary hypertension
资金
- NHLBI NIH HHS [R01 HL130307] Funding Source: Medline
Systemic sclerosis (SSc) is a rare and complex immune-mediated connective tissue disease characterized by multi-organ fibrosis and dysfunction. Systemic sclerosis-associated pulmonary arterial hypertension (SSc-PAH) is a leading cause of death in this population. Pulmonary arterial hypertension (PAH) can coexist with other forms of pulmonary hypertension in SSc, including pulmonary hypertension related to left heart disease, interstitial lung disease, chronic thromboembolism and pulmonary venous occlusive disease, which further complicates diagnosis and management. Available pulmonary arterial hypertension therapies target the nitric oxide, endothelin and prostacyclin pathways. These therapies have been studied in SSc-PAH in addition to idiopathic PAH, often with different treatment responses. In this article, we discuss the management as well as the treatment options for patients with SSc-PAH.
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