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Pulmonary arterial hypertension in systemic sclerosis: diagnostic pathway and therapeutic approach
  1. L Robertson1,
  2. A Pignone2,
  3. O Kowal-Bielecka3,
  4. G Fiori2,
  5. C P Denton4,
  6. M Matucci-Cerinic2
  1. 1Rheumatology Unit, Royal Cornwall Hospital, Truro, Cornwall, UK
  2. 2Department of Medicine, Division of Rheumatology, University of Florence, Florence, Italy
  3. 3Department of Rheumatology and Internal Diseases, Medical University of Bialystok, Bialystok, Poland
  4. 4Division of Rheumatology, Royal Free Hospital, London, UK
  1. Correspondence to:
    Professor M Matucci-Cerinic
    Department of Medicine, Section of Rheumatology, Villa Monna Tessa, Viale Pieraccini 18, 50122 Firenze, Italy;

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Case 1

A 63 year old white woman was admitted to hospital in February 2000 owing to rapidly increasing dyspnoea on exertion (NYHA III), chest pain, and fever. The presence of sclerodactyly with loss of distal parts of the fingers, and megacapillaries at capillaroscopy, allowed the diagnosis of limited cutaneous systemic sclerosis (fig 1). Physical examination also disclosed crackles in the lower lobes of the lungs, systolic murmur with loud pulmonary second sound, and feet oedema.

Figure 1

 Case 1. (A) Chest x ray findings show bibasilar reticular opacities, cardiomegaly with accentuation of the right ventricle, and enlargement of the pulmonary artery, which are consistent with interstitial lung disease and pulmonary hypertension. (B) High resolution CT of the lungs discloses features of interstitial lung disease with “ground glass” opacities and widening of the central blood vessels. (C) Doppler echocardiography shows blood regurgitation through the tricuspid valve, which allows estimation of PASP. (D) Perfusion scintigraphy of the lungs carried out in February 2000 (D/1) shows redistribution of the blood flow into the upper parts of the lungs and irregular areas of diminished blood flow in the peripheral parts of the lungs consistent with PAH and pulmonary thromboembolism. For comparison, perfusion scintigraphy of the lungs carried out in June 2000 (D/2) shows a significant increase in blood flow in the lower parts of both lungs, which paralleled a decrease in PASP as measured with Doppler echocardiography.

Laboratory tests included high erythrocyte sedimentation rate (145 mm/1st h), C reactive protein (2530 mg/l, normal <100 mg/l), presence of antinuclear antibodies (1/1280 and speckled pattern on indirect immunofluorescence), mild anaemia and thrombocytopenia (platelet count of 115×109/l), decreased arterial oxygen and carbon dioxide pressures. A chest x ray examination showed cardiomegaly, dilatation of the main pulmonary artery (fig 1), centralisation of pulmonary vascular flow, and reticular opacities in …

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