肺高血圧を伴ったCREST症候群の1例  [in Japanese] CREST Syndrome Associated with Pulmonary Hypertension  [in Japanese]

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Abstract

52歳の女性。初診の10年前に,顔面,頚部,四肢の色素沈着,5,6年前に,手指の腫脹,硬化,屈曲困難,1年前に,Raynaud症状が出現。8年前より胸部X線にて心拡大を指摘。6ヵ月前に,労作時息切れ,心胸郭比63.2%,肝腫大等の心不全症状が出現。10日前より起座呼吸となり,2001年1月15日,当院内科入院。心エコーで肺動脈収縮期圧81.4mmHgと著明高値であり,肺高血圧症(PH)の診断。抗核抗体5120倍,抗セントロメア抗体陽性。嚥下困難と顔面,頚部,前胸部の毛細血管拡張,両前腕,右腰部に皮下石灰沈着があり,PHを伴ったCREST症候群と診断。肺線維症はないが,肺拡散能は低下し,酸素吸入療法等の対症療法に不応で,生命予後は不良と思われる。自験例は皮膚症状が先行していたが,PHの症状が出現して8年目に初めてCREST症候群の診断がなされた。CREST症候群でもMCTDと同様, PHを伴うことが多く,致命的であるため,皮膚症状や合併症の早期発見が重要である。

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

  • The Nishinihon Jourrnal of Dermatology  

    The Nishinihon Jourrnal of Dermatology 64(2), 161-166, 2002-04-01 

    Western Division of Japanese Dermatological Association

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