CREST Syndrome Associated with Pulmonary Hypertension.

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  • 肺高血圧を伴ったＣＲＥＳＴ症候群の１例

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Abstract

A 52-year-old Japanese female who had a 10-year history of pigmentation of face, neck and extremities, a 5-year history of sclerodactyly, and one-year history of Raynaud’s phenomenon presented with an exacerbation of her symptoms of respiratory insufficiency and right heart failure in January 2001. An echocardiogram revealed severe pulmonary hypertension (PH) (systolic pressure of pulmonary artery 81.4mmHg) and tricuspid regurgitation. The laboratory findings were positive for antinuclear antibody (1 in 5120) and anticentromere antibody. She also had mild dysphagia, telangiectasia on her face, neck and chest, and subcutaneous calcinosis in the right lumber region and bilateral forearms. The diagnosis of CREST syndrome associated with PH was made based on those characteristics. Treatments with diuretics, vasodilators and oxygen via a nasal cannula could not releave her symptoms of PH including a decreased diffusing capacity although there was no pulmonary fibrosis. In our case, the symptoms of PH appeared long after the onset of telangiectasia, calcinosis and sclerodactyly, but the diagnosis of CREST syndrome was made only at the first visit to our clinic. The frequency of PH in CREST syndrome is as high as that in MCTD, and the prognosis is very poor. It is important to identify skin symptoms and complications in the early stage of CREST syndrome.

Journal

  • Nishi Nihon Hifuka

    Nishi Nihon Hifuka 64 (2), 161-166, 2002

    Western Division of Japanese Dermatological Association

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