Pulmonary hypertension in polymyositis-dermatomyositis: clinical and hemodynamic characteristics and response to vasoactive therapy

Abstract

The increased incidence of pulmonary hypertension and its association with decreased survival is well-recognised in patients with systemic sclerosis. This association is not widely appreciated in patients with polymyositis-dermatomyositis. We report clinical and hemodynamic characteristics and response to vasoactive therapy in three patients with polymyositis-dermatomyositis and pulmonary hypertension and discuss them in light of the available literature.

Keywords

connective tissue disease interstitial lung disease polymyositis-dermatomyositis pulmonary hypertension therapy

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