A CASE OF AUTOIMMUNE PULMONARY ALVEOLAR PROTEINOSIS WITH UNDERLYING MONOCLONAL B-CELL LYMPHOCYTOSIS
نویسندگان
چکیده
منابع مشابه
Rituximab therapy in autoimmune pulmonary alveolar proteinosis.
Idiopathic pulmonary alveolar proteinosis is presumed to be an autoimmune disorder that may lead to pulmonary insufficiency. However, steroids do not appear to be effective and the standard of therapy is whole-lung lavage. We report the first case of successful therapy with rituximab, which addresses the pathogenic mechanism of pulmonary alveolar proteinosis.
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概要 症例例,47才,男 性,教 員.主 訴 は,労 作時息切れ,咳 殿,喀 痰.28才 頃 より慢性気管支炎 と 言われていたが,最 近息切 れが増強 し受診 した.理 学 的所見では,胸 部で両下肺野後面に少数 の捻髪 音が聴 かれ,軽 度のばち状指趾が認め られた.胸 藻写真 で例,差 右両下肺野 に淡い微細粒状影 を,呼 吸機能検査 で例,拘 束性障害 と拡散能力の低 下を,動 脈血 ガス分析では,低 酸素血症 と過換気 の状態 を認 めた 。開胸肺生検に より組織学的に肺胞蛋 白症 と診断 された.L-シ ス チン内服で若 干の改善が認 め られ たが,Ramirez-Rら の方法 に準 じて病変の高度な左肺 の肺洗浄を施行 し,自 覚的 および 他覚的 症状 例著明に改筈 された.肺 洗浄液 の分析 で例,蛋 白 と燐脂質は正常 であ り,燐 脂質で例 レシチ ンと スフ...
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ژورنال
عنوان ژورنال: Chest
سال: 2020
ISSN: 0012-3692
DOI: 10.1016/j.chest.2020.08.1011