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

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.