Journal
INTEGRATED BLOOD PRESSURE CONTROL
Volume 13, Issue -, Pages 15-29Publisher
DOVE MEDICAL PRESS LTD
DOI: 10.2147/IBPC.S232038
Keywords
systemic sclerosis; scleroderma; pulmonary arterial hypertension; pulmonary hypertension
Categories
Funding
- NHLBI NIH HHS [R01 HL130307] Funding Source: Medline
Ask authors/readers for more resources
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.
Authors
I am an author on this paper
Click your name to claim this paper and add it to your profile.
Reviews
Recommended
No Data Available