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Year : 2017  |  Volume : 12  |  Issue : 6  |  Page : 204-210

Screening and management of pulmonary arterial hypertension in systemic sclerosis

Department of Medicine, University of Scleroderma Program, University of Michigan, Ann Arbor, MI, USA

Correspondence Address:
Vivek Nagaraja
Department of Medicine, Division of Rheumatology, University of Michigan, 300 North Ingalls Building, Room 7C27, Ann Arbor, MI 48109-5422
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0973-3698.219085

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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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