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PICTURES IN CLINICAL MEDICINE
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CREST Syndrome with Pulmonary Arterial Hypertension Yusuke Fukuda, Shin-ichiro Miura and Keijiro Saku Key words: CREST syndrome, pulmonary arterial hypertension (Intern Med 51: 441-442, 2012) (DOI: 10.2169/internalmedicine.51.6561)
Picture 1.
A 79-year-old woman was admitted to our hospital with effort dyspnea. Plain radiographs of the chest showed an enlargement of the heart and pleural fluid (Picture 1). Echocardiography showed remarkable right heart loading (Picture 2). Pulmonary arterial hypertension (PAH) and right heart failure manifesting hepatomegaly, ascites, jugular venous congestion, and pretibial pitting edema were observed. PAH was characterized by remarkable pulmonary hypertension with a normal pulmonary capillary wedge pressure assessed by Swan-Ganz catheterization. She had Raynaud’s phenomenon, sclerodactyly, telangiectasia about 10 years previously. Calcinosis circumscripta was found around the fingers (Picture 3, arrows). Antinuclear and anti-centromere antibodies were revealed to be 320 fold. We diagnosed the patient as CREST (calcinosis, Raynaud phenomenon, esophageal motility disorders, sclerodactyly, and telangiecta-
Picture 2.
Department of Cardiology, Fukuoka University School of Medicine, Japan Received for publication September 14, 2011; Accepted for publication October 4, 2011 Correspondence to Dr. Yusuke Fukuda,
[email protected]
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Intern Med 51: 441-442, 2012
DOI: 10.2169/internalmedicine.51.6561
Picture 3.
sia) syndrome resulting in PAH. PAH has a very poor prognosis, though CREST syndrome without PAH is a benign variant of progressive systemic sclerosis (1).
Reference
The authors state that they have no Conflict of Interest (COI).
1. Mathai SC, Hassoun PM. Pulmonary arterial hypertension associated with systemic sclerosis. Expert Rev Respir Med 5: 267-279, 2011.
Ⓒ 2012 The Japanese Society of Internal Medicine http://www.naika.or.jp/imindex.html
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