Journal
RHEUMATIC DISEASE CLINICS OF NORTH AMERICA
Volume 41, Issue 3, Pages 489-+Publisher
W B SAUNDERS CO-ELSEVIER INC
DOI: 10.1016/j.rdc.2015.04.009
Keywords
Systemic sclerosis; Pulmonary arterial hypertension; Monitoring; Diagnosis
Categories
Funding
- Gilead
- United Therapeutics
- Pfizer
- Actelion
- Scleroderma Research Foundation
- Scleroderma Foundation
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Pulmonary arterial hypertension (PAH) is one of the leading causes of death in patients with systemic sclerosis (SSc). Given the high prevalence and poor survival of SSc-PAH, and that aggressive management of mild disease may be associated with better outcomes, screening is critical. Right heart catheterization (RHO) is the gold standard for the definitive diagnosis of PAH, and should be performed in those patients in whom this diagnosis is suspected. Once a diagnosis of PAH is confirmed by RHO, treatment with PAH-specific therapies should be initiated as soon as possible.
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