Diagnosis of Pulmonary Arterial Hypertension in a Patient With Systemic Sclerosis

Jörg HW Distler, MD; Marius M Hoeper, MD; Oliver Distler, MD


Nat Clin Pract Rheumatol. 2008;4(3):160-164. 

In This Article

Treatment and Management

The natural disease course of PAH in patients with SSc has poor outcomes (Figure 1). A retrospective cohort study found a 1-year survival rate of only 55% in such patients, which was even worse than that in other forms of PAH[17] and much worse than that in patients with SSc who did not have lung disease.[18]

Chest X-rays of a patient with systemic sclerosis and end-stage pulmonary arterial hypertension. Arrows indicate dilatation of the right atrium (A) and right ventricle (B). Permission obtained from the European League Against Rheumatism ©; the X-rays were originally published in a module on connective tissue diseases for the European League Against Rheumatism online course on rheumatic diseases (www.eular-onlinecourse.org).

A number of specific treatments have become available for patients with PAH, including prostaglandin-derivatives (epoprostenol, treprostinil, beraprost and iloprost), endothelin-receptor antagonists (bosentan, sitaxsentan and ambrisentan), and the phosphodiesterase-type-5 inhibitor sildenafil. These therapies have been shown to improve hemodynamics, exercise capacity and quality of life in open and randomized controlled trials.[3,19] For patients with NYHA functional class III PAH, the available oral treatments (endothelin-receptor antagonists and sildenafil) are the therapeutic agents prescribed most frequently. No order of preference in these drugs could be definitively concluded from published studies. Rational therapeutic decisions must be made on the basis of the individual patient's specific situation, physician clinical judgment, and patient preferences. Treatment with prostaglandin derivates is an option if administration is feasible and the patient is compliant. On the basis of the current level of evidence, intravenous epoprostenol and inhaled iloprost are preferred over intravenous and inhaled treprostinil. Combination therapies can be considered in the case of treatment failure, but such patients should be included in clinical trials and registries. For current evidence-based treatment recommendations please refer to the latest American College of Chest Physicians guidelines.[4] It has to be emphasized that many of these recommendations are based on clinical trials in which patients with SSc or other connective tissue diseases were only a subgroup of the study cohort; the majority of patients suffered from idiopathic PAH. Recent data indicate that, compared to historical control treatments, bosentan and sitaxsentan improve survival in patients with PAH associated with SSc.[20]

Although there is no formal evidence of efficacy in randomized controlled trials, anticoagulation treatment is often used in patients with PAH in order to avoid local thrombosis, since severely disturbed blood flow in small pulmonary vessels, as well as an activated coagulation system, are associated with this disease.


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