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Review
. 2010 Feb;12(1):8-18.
doi: 10.1007/s11926-009-0078-1.

Diagnosis and management of pulmonary hypertension in systemic sclerosis

Nadera J Sweiss  1 , Linda Hushaw, Thenappan Thenappan, Ray Sawaqed, Roberto F Machado, Amit R Patel, Mardi Gomberg-Maitland, Aliya N Husain, Stephen L Archer
Affiliations
Review

Diagnosis and management of pulmonary hypertension in systemic sclerosis

Nadera J Sweiss et al. Curr Rheumatol Rep. 2010 Feb.

Abstract

Patients with systemic sclerosis (SSc) can develop pulmonary hypertension (PH; mean pulmonary artery pressure >/= 25 mm Hg) caused by pulmonary arterial hypertension (PAH), left ventricular disease, or pulmonary fibrosis. PAH is a pulmonary vascular disease, the diagnosis of which requires pulmonary capillary wedge pressure less than 15 mm Hg, pulmonary vascular resistance greater than 3 Wood Units, and exclusion of thromboembolism and parenchymal lung disease. Molecular mechanisms underlying PAH-SSc include activation of inflammatory and fibrogenic pathways in the vasculature and right ventricle. Circulating autoantibodies trigger endothelial damage and fibroblast activation. PAH most commonly occurs as a late complication in patients with limited cutaneous disease and anticentromere antibodies. Although echocardiography is a useful screening tool, heart catheterization is required to diagnose PAH before initiating therapy. Prognosis and therapeutic response are worse in PAH-SSc than in other PAH categories (median survival, 1-3 y). Approved therapies include prostacyclins, endothelin antagonists, and phosphodiesterase type 5 inhibitors. Research is needed to define disease mechanisms and develop effective therapies.

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Conflict of interest statement

Disclosure Dr. Archer reports no potential conflicts of interest relevant to this article.

Figures

Fig. 1
Fig. 1
Algorithm for early diagnosis of pulmonary arterial hypertension in patients with scleroderma. Concentric medial hypertrophy (a) and intimal fibrosis (b) can be seen in small pulmonary artery from a scleroderma lung. BNP B-type natriuretic peptide, CCBs calcium channel blockers, DLCO diffusing capacity of carbon monoxide, ECG electrocardiogram, ETRA endothelin receptor antagonist, FVC forced expiratory vital capacity, lcSSc limited cutaneous systemic sclerosis, mPAP mean pulmonary artery pressure, PDE-5 inhibitor phosphodiesterase type 5 inhibitor, PE pulmonary embolism, PF pulmonary fibrosis, RV right ventricular, RVH RV hypertrophy, TAPSE tricuspid annular plane systolic excursion, TR tricuspid regurgitation, V/Q ventilation/perfusion, WHO World Health Organization, WU Wood Units
See this image and copyright information in PMC

References

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