Effective immunosuppressive therapy in a patient with primary pulmonary hypertension

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The case history is described of a young woman who presented with primary pulmonary hypertension and non-specific inflammatory signs. The patient received prolonged immunosuppressive treatment with low dose methotrexate and prednisone without any vasodilator agent. After one year the pulmonary artery pressure fell from a mean value of 47 mm Hg to 30 mm Hg and there was a corresponding clinical response. This case suggests that, in patients with pulmonary hypertension of unknown origin, immunopathogenetic factor should be sought in order to consider the utility of immunosuppressive therapy.

(Thorax 1999;54:372-374)

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