Severe Pulmonary Hypertension Associated with Primary Sjogren's Syndrome

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タイトル別名
  • Severe Pulmonary Hypertension Associated with Primary Sjoegren's Syndrome
  • Autoimmune Aspects of Pulmonary Hypertension in Collagen Vascular Diseases

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説明

Severe pulmonary hypertension is one of the fetal complications in various connective tissue diseases. We report a case of severe pulmonary hypertension associated with primary Sjögren's syndrome. In a lung biopsy specimen, there were findings of intimal and medial hypertrophy with narrowing vessel lumina and plexiform lesions. Moreover, deposits of immunoglobulin M, immunoglobulin A and complement protein Clq were found in the pulmonary arterial walls. Although pulmonary hypertension was refractory to oral prostacyclin, steroid therapy improved the clinical and hemodynamic conditions. In the present case, the immunological etiology may be related to the mechanisms of pulmonary hypertension associated with Sjögren's syndrome.<br>(Internal Medicine 42: 1248-1252, 2003)

収録刊行物

  • Internal Medicine

    Internal Medicine 42 (12), 1248-1252, 2003

    一般社団法人 日本内科学会

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