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