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嗜酸(suan)性(xing)細(xi)胞(bao)性(xing)肺炎是一組病因(yin)明(ming)確或尚未明(ming)確，以嗜酸(suan)性(xing)細(xi)胞(bao)浸潤(run)為特點(dian)，常伴周圍血嗜酸(suan)細(xi)胞(bao)增多(duo)的(de)疾病。有(you)時(shi)稱為嗜酸(suan)性(xing)細(xi)胞(bao)增多(duo)性(xing)肺浸潤(run)(PIE)綜合征。

基本概述

嗜(shi)酸(suan)性細胞性肺炎是一組病因(yin)明確或尚未明確，以嗜(shi)酸(suan)性細胞浸潤為特點，常伴(ban)周(zhou)圍血嗜(shi)酸(suan)細胞增多的疾病.

有時稱為嗜酸性細(xi)胞增多性肺浸潤(run)（PIE）綜合征。

病因學和發病機制

病(bing)因(yin)(yin)包(bao)括寄生蟲(chong)（如(ru)蛔蟲(chong)，弓蛔蚴蟲(chong)和絲蟲(chong)），藥物（如(ru)青霉素，對氨基水(shui)楊酸(suan)(suan)，肼苯噠嗪，呋喃(nan)妥(tuo)因(yin)(yin)，氯(lv)丙嗪，磺胺制劑）；化(hua)學過敏(min)物質（如(ru)蒸氣形式(shi)吸(xi)入的碳化(hua)鎳）；和真菌（如(ru)煙曲霉，它引(yin)起變應性(xing)支氣管(guan)肺曲霉病(bing)見后述）.盡管(guan)疑有過敏(min)性(xing)機制，但大多數嗜酸(suan)(suan)性(xing)細胞肺炎病(bing)因(yin)(yin)不明.嗜酸(suan)(suan)性(xing)細胞增多提示為(wei)(wei)第(di)Ⅰ型(xing)(xing)過敏(min)反應，綜合征(zheng)的其他(ta)特點（血管(guan)炎，圓細胞浸潤）提示為(wei)(wei)第(di)Ⅲ型(xing)(xing)亦(yi)可能為(wei)(wei)第(di)Ⅳ型(xing)(xing)反應.

嗜(shi)酸性(xing)(xing)(xing)(xing)(xing)細(xi)(xi)胞(bao)性(xing)(xing)(xing)(xing)(xing)肺(fei)炎(yan)（表(biao)76-3）常合(he)(he)并有(you)支氣管(guan)哮(xiao)喘(chuan)(chuan).合(he)(he)并哮(xiao)喘(chuan)(chuan)及(ji)病因不明的嗜(shi)酸性(xing)(xing)(xing)(xing)(xing)細(xi)(xi)胞(bao)性(xing)(xing)(xing)(xing)(xing)肺(fei)炎(yan)可分為三(san)類：外源性(xing)(xing)(xing)(xing)(xing)支氣管(guan)哮(xiao)喘(chuan)(chuan)伴PIE綜(zong)(zong)合(he)(he)征(zheng)(zheng)，事實(shi)上(shang)，常為變應(ying)性(xing)(xing)(xing)(xing)(xing)支氣管(guan)肺(fei)曲(qu)霉(mei)病；內源性(xing)(xing)(xing)(xing)(xing)支氣管(guan)哮(xiao)喘(chuan)(chuan)伴PIE綜(zong)(zong)合(he)(he)征(zheng)(zheng)（慢性(xing)(xing)(xing)(xing)(xing)嗜(shi)酸性(xing)(xing)(xing)(xing)(xing)細(xi)(xi)胞(bao)性(xing)(xing)(xing)(xing)(xing)肺(fei)炎(yan)），胸片上(shang)常出現特征(zheng)(zheng)性(xing)(xing)(xing)(xing)(xing)的肺(fei)周圍部浸潤；和變應(ying)性(xing)(xing)(xing)(xing)(xing)肉芽腫病（Churg-Strauss綜(zong)(zong)合(he)(he)征(zheng)(zheng)，一種結節性(xing)(xing)(xing)(xing)(xing)多(duo)關節炎(yan)伴肺(fei)病變）.單(dan)純性(xing)(xing)(xing)(xing)(xing)嗜(shi)酸性(xing)(xing)(xing)(xing)(xing)細(xi)(xi)胞(bao)性(xing)(xing)(xing)(xing)(xing)肺(fei)炎(yan)（LÖffler綜(zong)(zong)合(he)(he)征(zheng)(zheng)）偶可合(he)(he)并哮(xiao)喘(chuan)(chuan).

不(bu)(bu)伴哮喘(chuan)的(de)(de)嗜(shi)(shi)(shi)(shi)(shi)(shi)酸(suan)性(xing)(xing)(xing)(xing)(xing)(xing)細胞(bao)(bao)(bao)(bao)性(xing)(xing)(xing)(xing)(xing)(xing)肺(fei)炎(yan)包括(kuo)急性(xing)(xing)(xing)(xing)(xing)(xing)嗜(shi)(shi)(shi)(shi)(shi)(shi)酸(suan)性(xing)(xing)(xing)(xing)(xing)(xing)細胞(bao)(bao)(bao)(bao)性(xing)(xing)(xing)(xing)(xing)(xing)肺(fei)炎(yan)，嗜(shi)(shi)(shi)(shi)(shi)(shi)酸(suan)性(xing)(xing)(xing)(xing)(xing)(xing)細胞(bao)(bao)(bao)(bao)性(xing)(xing)(xing)(xing)(xing)(xing)肌(ji)痛綜合(he)征(zheng)和(he)(he)嗜(shi)(shi)(shi)(shi)(shi)(shi)酸(suan)性(xing)(xing)(xing)(xing)(xing)(xing)細胞(bao)(bao)(bao)(bao)增多(duo)(duo)綜合(he)征(zheng).急性(xing)(xing)(xing)(xing)(xing)(xing)細胞(bao)(bao)(bao)(bao)性(xing)(xing)(xing)(xing)(xing)(xing)肺(fei)炎(yan)是一(yi)種(zhong)原(yuan)因(yin)不(bu)(bu)明(ming)的(de)(de)不(bu)(bu)同病種(zhong)，可引(yin)起(qi)急性(xing)(xing)(xing)(xing)(xing)(xing)發(fa)熱(re)，嚴重低氧血癥，彌漫性(xing)(xing)(xing)(xing)(xing)(xing)肺(fei)浸(jin)潤以及支氣(qi)管肺(fei)泡灌洗液(ye)中的(de)(de)嗜(shi)(shi)(shi)(shi)(shi)(shi)酸(suan)性(xing)(xing)(xing)(xing)(xing)(xing)細胞(bao)(bao)(bao)(bao)比例>25%.經皮質激素(su)治(zhi)療可迅速，徹底緩(huan)解(jie).嗜(shi)(shi)(shi)(shi)(shi)(shi)酸(suan)性(xing)(xing)(xing)(xing)(xing)(xing)細胞(bao)(bao)(bao)(bao)性(xing)(xing)(xing)(xing)(xing)(xing)肌(ji)痛綜合(he)征(zheng)與食用大量的(de)(de)作為(wei)食物添(tian)加劑的(de)(de)L-色氨(an)酸(suan)有(you)(you)關.肺(fei)浸(jin)潤偶(ou)可伴有(you)(you)肌(ji)痛，肌(ji)無力(li)，皮疹和(he)(he)類似于硬皮病的(de)(de)軟組(zu)織硬結.嗜(shi)(shi)(shi)(shi)(shi)(shi)酸(suan)性(xing)(xing)(xing)(xing)(xing)(xing)細胞(bao)(bao)(bao)(bao)增多(duo)(duo)綜合(he)征(zheng)的(de)(de)診斷標準為(wei)持(chi)續性(xing)(xing)(xing)(xing)(xing)(xing)嗜(shi)(shi)(shi)(shi)(shi)(shi)酸(suan)性(xing)(xing)(xing)(xing)(xing)(xing)細胞(bao)(bao)(bao)(bao)增多(duo)(duo)（>1500/mm3）達6個月以上(shang)，缺乏(fa)其(qi)他引(yin)起(qi)嗜(shi)(shi)(shi)(shi)(shi)(shi)酸(suan)性(xing)(xing)(xing)(xing)(xing)(xing)細胞(bao)(bao)(bao)(bao)增多(duo)(duo)的(de)(de)病因(yin)，累及心，肝，脾，中樞神經系(xi)統或肺(fei)，心臟最(zui)常受(shou)累.發(fa)熱(re)，體重減輕(qing)和(he)(he)貧血也常見,常可發(fa)生動脈(mo)而非靜脈(mo)性(xing)(xing)(xing)(xing)(xing)(xing)血栓栓塞疾病。

特征(zheng)性改變包括(kuo)嗜酸(suan)性細(xi)(xi)胞(bao)(bao)，大單(dan)核細(xi)(xi)胞(bao)(bao)充滿肺泡，肺泡間(jian)隔內嗜酸(suan)性細(xi)(xi)胞(bao)(bao)，漿細(xi)(xi)胞(bao)(bao)和大，小單(dan)核細(xi)(xi)胞(bao)(bao)浸潤.也可有(you)細(xi)(xi)支氣管內粘液填塞和血管浸潤。

癥狀和體征

癥(zheng)狀(zhuang)和體征(zheng)可(ke)輕(qing)微，也可(ke)危(wei)及(ji)生(sheng)命.可(ke)伴低度(du)發(fa)熱，輕(qing)度(du)（如有(you)(you)）呼(hu)(hu)吸(xi)道癥(zheng)狀(zhuang)，可(ke)即時(shi)恢復(fu).其他(ta)類型的PIE綜合(he)征(zheng)，可(ke)有(you)(you)發(fa)熱和支(zhi)氣管(guan)哮喘(chuan)癥(zheng)狀(zhuang)，包括咳嗽，喘(chuan)鳴和靜息時(shi)呼(hu)(hu)吸(xi)困難.如不治療，慢(man)性(xing)嗜(shi)酸性(xing)細胞性(xing)肺炎(yan)(yan)常進行性(xing)發(fa)展至危(wei)及(ji)生(sheng)命，類似急性(xing)嗜(shi)酸性(xing)肺炎(yan)(yan).常有(you)(you)明顯增高的嗜(shi)酸性(xing)細胞（20%~40%，有(you)(you)時(shi)更高）.胸部X線表現(xian)為不同(tong)肺葉內(nei)有(you)(you)迅速出現(xian)及(ji)迅速消失(shi)的浸潤(run)病灶（游(you)走性(xing)浸潤(run)）。

臨床診斷

根據(ju)病(bing)人居住(zhu)的地理區域尋(xun)找(zhao)致病(bing)蠕蟲(chong)，痰內可找(zhao)到(dao)寄(ji)生(sheng)蟲(chong)和(he)(he)煙(yan)曲霉(mei)，需仔細詢問用(yong)藥史.鑒別診(zhen)斷包(bao)括結核，結節病(bing)，霍奇金病(bing)和(he)(he)其(qi)他淋巴組織增生(sheng)性(xing)(xing)(xing)(xing)疾(ji)(ji)病(bing)，肺(fei)嗜酸性(xing)(xing)(xing)(xing)細胞(bao)性(xing)(xing)(xing)(xing)肉芽腫(zhong)，脫屑性(xing)(xing)(xing)(xing)間質性(xing)(xing)(xing)(xing)肺(fei)炎(yan)和(he)(he)膠原性(xing)(xing)(xing)(xing)血管(guan)疾(ji)(ji)病(bing)。過敏(min)性(xing)(xing)(xing)(xing)肺(fei)炎(yan)和(he)(he)Wegener肉芽腫(zhong)常不(bu)伴嗜酸性(xing)(xing)(xing)(xing)細胞(bao)增多。

治療

本病(bing)可(ke)為(wei)自限性，呈良性，可(ke)不需治療(liao).如癥狀嚴重，使用(yong)(yong)皮(pi)質類固醇常(chang)(chang)有(you)極好效(xiao)果；對于(yu)急性嗜(shi)酸性細胞(bao)肺炎和特發性慢性嗜(shi)酸性肺炎者該(gai)治療(liao)可(ke)挽(wan)救生命。如出(chu)現支(zhi)氣管哮(xiao)喘，采用(yong)(yong)通常(chang)(chang)治療(liao)方法.有(you)蠕蟲感染的，應使用(yong)(yong)驅蟲藥。