Screening and management of pulmonary arterial hypertension in systemic sclerosis
Systemic sclerosis-associated pulmonary hypertension (SSc-PH) and pulmonary arterial (PA) hypertension (SSc-PAH) are well-recognized manifestations. SSc-PH is a hemodynamic observation, and it is important to identify underlying etiology. SSc patients commonly have mixed etiology for SSc-PH due to i...
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| Main Authors: | , |
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| Format: | Article |
| Language: | English |
| Published: |
SAGE Publishing
2017-01-01
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| Series: | Indian Journal of Rheumatology |
| Subjects: | |
| Online Access: | http://www.indianjrheumatol.com/article.asp?issn=0973-3698;year=2017;volume=12;issue=6;spage=204;epage=210;aulast=Nagaraja |
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| Summary: | Systemic sclerosis-associated pulmonary hypertension (SSc-PH) and pulmonary arterial (PA) hypertension (SSc-PAH) are well-recognized manifestations. SSc-PH is a hemodynamic observation, and it is important to identify underlying etiology. SSc patients commonly have mixed etiology for SSc-PH due to interstitial lung disease, PAH, and left heart disease. SSc-PAH is associated with high morbidity and mortality. Early detection of PAH through routine screening improves survival in patients with SSc. Right heart catheterization is mandatory to diagnose PAH. SSc-PAH patients should be managed by a multidisciplinary team comprising of rheumatologist, pulmonologist, cardiologist, and physiotherapist. Various pharmacotherapy options to treat SSc-PAH are derived from the idiopathic PAH management. Upfront or sequential combination therapy of PAH-specific drugs seems to confer a clinical benefit compared to monotherapy. Cardiopulmonary rehabilitation should be considered as a part of the management plan. Lung transplantation is a consideration in patients who are not responding to pharmacotherapy. Although the long-term prognosis of SSc-PAH has been historically poor, the landscape is gradually changing with early detection and institution of treatment. |
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| ISSN: | 0973-3698 0973-3701 |