© 2005 by BMJ Publishing Group Ltd & European League Against Rheumatism
LESSON OF THE MONTH
Pulmonary arterial hypertension in systemic sclerosis: diagnostic pathway and therapeutic approach
Series editor: Anthony D Woolf
1 Rheumatology Unit, Royal Cornwall Hospital, Truro, Cornwall, UK
2 Department of Medicine, Division of Rheumatology, University of Florence, Florence, Italy
3 Department of Rheumatology and Internal Diseases, Medical University of Bialystok, Bialystok, Poland
4 Division of Rheumatology, Royal Free Hospital, London, UK
Correspondence to:
Correspondence to:
Professor M Matucci-Cerinic
Department of Medicine, Section of Rheumatology, Villa Monna Tessa, Viale Pieraccini 18, 50122 Firenze, Italy; cerinic@unifi.it
Accepted 22 December 2004
Abbreviations: ACE, angiotensin converting enzyme; CYC, cyclophosphamide; ECG, electrocardiography; FVC, forced vital capacity; PAH, pulmonary artery hypertension; PASP, pulmonary artery systolic pressure; SSC, systemic sclerosis
Keywords: pulmonary arterial hypertension; systemic sclerosis
| The first 150 words of the full text of this article appear below. |
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.
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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 | |||||||||
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