Diagnosis and management of pulmonary hypertension in systemic sclerosis

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

Research output: Contribution to journalReview article

21 Citations (Scopus)

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.

Original languageEnglish (US)
Pages (from-to)8-18
Number of pages11
JournalCurrent rheumatology reports
Volume12
Issue number1
DOIs
StatePublished - Feb 1 2010

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Systemic Scleroderma
Pulmonary Hypertension
Lung Diseases
Prostaglandins I
Phosphodiesterase 5 Inhibitors
Pulmonary Wedge Pressure
Pulmonary Fibrosis
Thromboembolism
Therapeutics
Cardiac Catheterization
Vascular Diseases
Skin Diseases
Vascular Resistance
Autoantibodies
Pulmonary Artery
Heart Ventricles
Echocardiography
Fibroblasts
Pressure
Survival

Keywords

  • Circulating autoantibodies
  • Connective tissue disease
  • Endothelin receptor antagonists
  • Flolan (epoprostenol)
  • Phosphodiesterase 5 inhibitors
  • Pulmonary arterial hypertension

ASJC Scopus subject areas

  • Rheumatology

Cite this

Sweiss, N. J., Hushaw, L., Thenappan, T., Sawaqed, R., MacHado, R. F., Patel, A. R., ... Archer, S. L. (2010). Diagnosis and management of pulmonary hypertension in systemic sclerosis. Current rheumatology reports, 12(1), 8-18. https://doi.org/10.1007/s11926-009-0078-1

Diagnosis and management of pulmonary hypertension in systemic sclerosis. / Sweiss, Nadera J.; Hushaw, Linda; Thenappan, Thenappan; Sawaqed, Ray; MacHado, Roberto F.; Patel, Amit R.; Gomberg-Maitland, Mardi; Husain, Aliya N.; Archer, Stephen L.

In: Current rheumatology reports, Vol. 12, No. 1, 01.02.2010, p. 8-18.

Research output: Contribution to journalReview article

Sweiss, NJ, Hushaw, L, Thenappan, T, Sawaqed, R, MacHado, RF, Patel, AR, Gomberg-Maitland, M, Husain, AN & Archer, SL 2010, 'Diagnosis and management of pulmonary hypertension in systemic sclerosis', Current rheumatology reports, vol. 12, no. 1, pp. 8-18. https://doi.org/10.1007/s11926-009-0078-1
Sweiss, Nadera J. ; Hushaw, Linda ; Thenappan, Thenappan ; Sawaqed, Ray ; MacHado, Roberto F. ; Patel, Amit R. ; Gomberg-Maitland, Mardi ; Husain, Aliya N. ; Archer, Stephen L. / Diagnosis and management of pulmonary hypertension in systemic sclerosis. In: Current rheumatology reports. 2010 ; Vol. 12, No. 1. pp. 8-18.
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