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Rheumatology Advance Access originally published online on June 28, 2008
Rheumatology 2008 47(9):1431-1432; doi:10.1093/rheumatology/ken241
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© The Author 2008. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For Permissions, please email: journals.permissions@oxfordjournals.org

Pulmonary artery hypertension as the presenting feature of systemic sclerosis sine scleroderma

J. D. Pauling1, H. Gunawardena1, J. G. Coghlan2, J. Easaw3, J. Suntharalingam4 and N. J. McHugh1

1Royal National Hospital for Rheumatic Diseases, Bath, 2Department of Cardiology, Royal Free Hospital, London, 3Department of Cardiology and 4Department of Respiratory Medicine, Royal United Hospital, Bath, UK

Correspondence to: N. J. McHugh, Royal National Hospital for Rheumatic Diseases, Bath, BA1 1RL, UK. E-mail: neil.mchugh@rnhrd.nhs.uk

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SIR, Pulmonary artery hypertension (PAH) is now the leading cause of mortality in patients with lcSSc. Effective treatments for PAH such as prostenoid analogues, endothelin receptor antagonists and phosphodiesterase inhibitors are currently available, and screening for pulmonary vascular disease is recommended for all patients with lcSSc. Major organ involvement as part of SSc without the characteristic skin changes of scleroderma, defined as SSc sine scleroderma (ssSSc), was first described in 1954 [1]. Here we report the case of a 29-yr-old . . . [Full Text of this Article]


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