COPD guidelines

Abstract

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 factors should be sought in order to consider the utility of immunosuppressive therapy. (Thorax 1999;54:372-374) Keywords: pulmonary hypertension; methotrexate; transforming growth factor Primary pulmonary hypertension (PPH) is a rare disease of unknown origin that should be suspected in any young patient who presents with unexplained eVort dyspnoea and electrocardiographic signs of right ventricular hypertrophy in the apparent absence of any underlying cause.1 Pulmonary hypertension clinically and histologically similar to PPH has been described in association with connective tissue diseases and other immunological disorders, also in the absence of any parenchymal lung involvement.2 Primary forms usually show a rapidly progressive course despite treatment, with a mean survival of 2.5 years. 3We describe a patient with PPH who, after treatment with immunosuppressive therapy, showed a clinical improvement. Case reportA 25 year old woman was admitted in December 1994 because of the recent onset (less than six months) of rest dyspnoea (NYHA class III), cough, cyanosis, hypotension (80/60 mm Hg), tachycardia (120-130 bpm), and congestive heart failure. She had a five year history of recurrent mild temperature elevation, asthenia, myalgia, arthralgia, transient diVuse lymphoadenomegaly, neutrophil leukocytosis, and persistent elevation of acute phase reactants, despite anti-inflammatory therapy. Tests for antinuclear (ANA and ENA) and antiphospholipid antibodies, rheumatoid factor, ANCA and other autoantibodies, HIV and other infectious agents were always negative. The patient had never been pregnant nor used appetite suppressants or any other drug potentially able to induce pulmonary hypertension. 1There were no signs suggestive of connective tissue disease.Laboratory tests gave the following results: erythrocyte sedimentation rate, 29 mm/h; fibrinogen, 5.32 g/l (normal 2.0-4.0 g/l); antithrombin III 1.42 IU/ml (normal 0.8-1.2); positive indirect antiglobulin test; serum IgE, 1.817 kU/l (normal <200) with negative RAST; serum IgG, 19.30 g/l (normal 8.0-15.0 g/l); serum IgA, 0.32 g/l (normal 1.00-4.90 g/l); ferritin, 1808 ng/l (normal 4-233 ng/l); positivity for HLA-DR4 antigen; moderate increase in peripheral blood CD8+ lymphocytes. Other routine tests were negative or normal.Lung scans with 99m-technetium labelled albumin macroaggregates and 133-xenon gas and deep vein Doppler ultrasonography of the lower extremities were normal. The electrocardiogram, which had been normal six months earlier, showed sig...