A relatively rare disease characterized primarily by repeated pulmonary hemorrhage and rapidly progressing glomerulonephritis. Its cause is unknown, but it is thought to be an autoimmune disease caused by antibodies (anti-basement membrane antibodies) common to the lungs and kidneys. It is generally seen in young men. It begins with bloody sputum and hemoptysis, and coughing and dyspnea are often also present. Renal symptoms such as proteinuria, hematuria, and edema appear several weeks to several months later. It is often preceded by cold-like symptoms. Chest X-rays show diffuse nodular or patchy shadows in both lungs, but repeated bleeding causes lung fibrosis to progress, resulting in linear or reticular shadows. Source: Heibonsha World Encyclopedia, 2nd Edition Information |
反復する肺出血と急速に進行する糸球体腎炎をおもな特徴とする比較的まれな病気。その原因は不明であるが,肺と腎臓に共通する抗体(抗基底膜抗体)によってひきおこされる自己免疫疾患であると考えられている。一般に若年男子にみられる。血痰や喀血で発症し,咳や呼吸困難などもしばしばみられる。数週~数ヵ月後にタンパク尿や血尿,浮腫などの腎臓症状があらわれる。しばしば風邪のような症状が先行する。胸部X線写真では,両側の肺に広範に瀰漫(びまん)性の小結節状,または斑状の陰影がみられるが,出血を繰り返すと肺の繊維化が進行し,線状や網状の影を呈する。
出典 株式会社平凡社世界大百科事典 第2版について 情報 |
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