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嗜酸(suan)性(xing)細胞性(xing)肺炎是一組病因明確或尚未(wei)明確，以嗜酸(suan)性(xing)細胞浸潤(run)為(wei)特點(dian)，常伴周圍血嗜酸(suan)細胞增多(duo)的疾病。有時稱為(wei)嗜酸(suan)性(xing)細胞增多(duo)性(xing)肺浸潤(run)(PIE)綜合征。

基本概述

嗜酸(suan)性細(xi)胞(bao)性肺(fei)炎是(shi)一(yi)組病因明確(que)或尚未(wei)明確(que)，以(yi)嗜酸(suan)性細(xi)胞(bao)浸潤為特點，常伴周圍血嗜酸(suan)細(xi)胞(bao)增多的疾病.

有時(shi)稱為嗜酸性(xing)(xing)細胞增(zeng)多性(xing)(xing)肺浸潤(run)（PIE）綜合征。

病因學和發病機制

病(bing)(bing)因包(bao)括(kuo)寄(ji)生蟲(chong)（如(ru)蛔蟲(chong)，弓(gong)蛔蚴蟲(chong)和絲蟲(chong)），藥(yao)物（如(ru)青霉素，對氨基水(shui)楊酸，肼(jing)苯噠嗪，呋(fu)喃妥因，氯丙(bing)嗪，磺(huang)胺制(zhi)劑）；化學(xue)過(guo)敏物質（如(ru)蒸(zheng)氣形式(shi)吸入的碳化鎳）；和真菌（如(ru)煙曲霉，它(ta)引(yin)起變應性(xing)支氣管肺(fei)曲霉病(bing)(bing)見后述）.盡(jin)管疑有(you)過(guo)敏性(xing)機制(zhi)，但大(da)多數嗜(shi)酸性(xing)細胞(bao)(bao)肺(fei)炎(yan)病(bing)(bing)因不(bu)明.嗜(shi)酸性(xing)細胞(bao)(bao)增多提(ti)(ti)示為第Ⅰ型(xing)(xing)過(guo)敏反應，綜(zong)合征的其他特點(dian)（血管炎(yan)，圓細胞(bao)(bao)浸潤）提(ti)(ti)示為第Ⅲ型(xing)(xing)亦可能為第Ⅳ型(xing)(xing)反應.

嗜(shi)酸(suan)性(xing)細胞性(xing)肺(fei)炎(yan)（表76-3）常合并有支(zhi)(zhi)氣(qi)(qi)(qi)管(guan)(guan)哮(xiao)(xiao)喘.合并哮(xiao)(xiao)喘及病(bing)因(yin)不明的(de)(de)嗜(shi)酸(suan)性(xing)細胞性(xing)肺(fei)炎(yan)可(ke)分為三類：外源(yuan)性(xing)支(zhi)(zhi)氣(qi)(qi)(qi)管(guan)(guan)哮(xiao)(xiao)喘伴(ban)PIE綜(zong)合征(zheng)(zheng)，事實上，常為變(bian)應(ying)性(xing)支(zhi)(zhi)氣(qi)(qi)(qi)管(guan)(guan)肺(fei)曲霉病(bing)；內(nei)源(yuan)性(xing)支(zhi)(zhi)氣(qi)(qi)(qi)管(guan)(guan)哮(xiao)(xiao)喘伴(ban)PIE綜(zong)合征(zheng)(zheng)（慢性(xing)嗜(shi)酸(suan)性(xing)細胞性(xing)肺(fei)炎(yan)），胸片上常出現(xian)特征(zheng)(zheng)性(xing)的(de)(de)肺(fei)周圍部(bu)浸潤；和變(bian)應(ying)性(xing)肉芽腫(zhong)病(bing)（Churg-Strauss綜(zong)合征(zheng)(zheng)，一種(zhong)結節(jie)性(xing)多(duo)關節(jie)炎(yan)伴(ban)肺(fei)病(bing)變(bian)）.單(dan)純性(xing)嗜(shi)酸(suan)性(xing)細胞性(xing)肺(fei)炎(yan)（LÖffler綜(zong)合征(zheng)(zheng)）偶可(ke)合并哮(xiao)(xiao)喘.

不(bu)伴(ban)(ban)哮喘的(de)(de)嗜酸(suan)性(xing)(xing)(xing)(xing)細(xi)胞(bao)(bao)(bao)性(xing)(xing)(xing)(xing)肺(fei)炎(yan)包括急性(xing)(xing)(xing)(xing)嗜酸(suan)性(xing)(xing)(xing)(xing)細(xi)胞(bao)(bao)(bao)性(xing)(xing)(xing)(xing)肺(fei)炎(yan)，嗜酸(suan)性(xing)(xing)(xing)(xing)細(xi)胞(bao)(bao)(bao)性(xing)(xing)(xing)(xing)肌痛(tong)綜(zong)合征和(he)嗜酸(suan)性(xing)(xing)(xing)(xing)細(xi)胞(bao)(bao)(bao)增多(duo)(duo)綜(zong)合征.急性(xing)(xing)(xing)(xing)細(xi)胞(bao)(bao)(bao)性(xing)(xing)(xing)(xing)肺(fei)炎(yan)是一種(zhong)(zhong)原因不(bu)明的(de)(de)不(bu)同病種(zhong)(zhong)，可(ke)(ke)引起(qi)急性(xing)(xing)(xing)(xing)發(fa)(fa)熱，嚴(yan)重低氧血癥，彌(mi)漫性(xing)(xing)(xing)(xing)肺(fei)浸潤以及支氣(qi)管肺(fei)泡灌洗液(ye)中的(de)(de)嗜酸(suan)性(xing)(xing)(xing)(xing)細(xi)胞(bao)(bao)(bao)比例>25%.經(jing)皮質激素(su)治療可(ke)(ke)迅速，徹底緩解.嗜酸(suan)性(xing)(xing)(xing)(xing)細(xi)胞(bao)(bao)(bao)性(xing)(xing)(xing)(xing)肌痛(tong)綜(zong)合征與食用大量的(de)(de)作為(wei)食物(wu)添加劑的(de)(de)L-色氨酸(suan)有(you)關(guan).肺(fei)浸潤偶可(ke)(ke)伴(ban)(ban)有(you)肌痛(tong)，肌無力，皮疹和(he)類似于硬皮病的(de)(de)軟組織硬結.嗜酸(suan)性(xing)(xing)(xing)(xing)細(xi)胞(bao)(bao)(bao)增多(duo)(duo)綜(zong)合征的(de)(de)診斷標(biao)準(zhun)為(wei)持續性(xing)(xing)(xing)(xing)嗜酸(suan)性(xing)(xing)(xing)(xing)細(xi)胞(bao)(bao)(bao)增多(duo)(duo)（>1500/mm3）達(da)6個月以上，缺乏其他引起(qi)嗜酸(suan)性(xing)(xing)(xing)(xing)細(xi)胞(bao)(bao)(bao)增多(duo)(duo)的(de)(de)病因，累及心(xin)，肝，脾，中樞神經(jing)系(xi)統(tong)或肺(fei)，心(xin)臟最常受累.發(fa)(fa)熱，體重減輕和(he)貧血也常見,常可(ke)(ke)發(fa)(fa)生動脈而(er)非(fei)靜脈性(xing)(xing)(xing)(xing)血栓栓塞疾病。

特征性(xing)改變包括嗜酸(suan)性(xing)細(xi)(xi)胞(bao)，大(da)單核細(xi)(xi)胞(bao)充滿肺(fei)泡，肺(fei)泡間(jian)隔內嗜酸(suan)性(xing)細(xi)(xi)胞(bao)，漿細(xi)(xi)胞(bao)和(he)大(da)，小單核細(xi)(xi)胞(bao)浸潤.也可有細(xi)(xi)支氣管內粘液填塞和(he)血管浸潤。

癥狀和體征

癥狀和(he)體征(zheng)可輕微，也(ye)可危及(ji)生命.可伴低度(du)發熱，輕度(du)（如有）呼(hu)吸(xi)道癥狀，可即時(shi)(shi)恢復.其他類型的PIE綜合征(zheng)，可有發熱和(he)支(zhi)氣管哮喘(chuan)癥狀，包括咳嗽，喘(chuan)鳴(ming)和(he)靜息時(shi)(shi)呼(hu)吸(xi)困(kun)難(nan).如不治療，慢性(xing)(xing)嗜(shi)酸性(xing)(xing)細胞(bao)(bao)性(xing)(xing)肺(fei)炎常(chang)進行性(xing)(xing)發展至危及(ji)生命，類似急性(xing)(xing)嗜(shi)酸性(xing)(xing)肺(fei)炎.常(chang)有明顯增高的嗜(shi)酸性(xing)(xing)細胞(bao)(bao)（20%~40%，有時(shi)(shi)更高）.胸部X線表現為不同肺(fei)葉內有迅速出現及(ji)迅速消失的浸(jin)潤(run)(run)病灶（游走性(xing)(xing)浸(jin)潤(run)(run)）。

臨床診斷

根據病(bing)(bing)人居住的地理區域(yu)尋找(zhao)致病(bing)(bing)蠕蟲，痰(tan)內可找(zhao)到寄生蟲和煙曲霉(mei)，需仔細(xi)詢問用藥(yao)史.鑒別診斷包(bao)括結核，結節病(bing)(bing)，霍奇金病(bing)(bing)和其他(ta)淋巴組(zu)織增生性(xing)(xing)(xing)疾(ji)病(bing)(bing)，肺(fei)嗜(shi)酸性(xing)(xing)(xing)細(xi)胞性(xing)(xing)(xing)肉芽(ya)(ya)腫，脫屑(xie)性(xing)(xing)(xing)間質(zhi)性(xing)(xing)(xing)肺(fei)炎(yan)和膠原性(xing)(xing)(xing)血管(guan)疾(ji)病(bing)(bing)。過敏性(xing)(xing)(xing)肺(fei)炎(yan)和Wegener肉芽(ya)(ya)腫常不伴嗜(shi)酸性(xing)(xing)(xing)細(xi)胞增多。

治療

本病可為自限性(xing)(xing)(xing)，呈良性(xing)(xing)(xing)，可不需治療(liao).如癥狀嚴重，使(shi)用皮質類固醇常有(you)極好效果；對于急性(xing)(xing)(xing)嗜(shi)酸性(xing)(xing)(xing)細胞(bao)肺(fei)炎和(he)特發性(xing)(xing)(xing)慢性(xing)(xing)(xing)嗜(shi)酸性(xing)(xing)(xing)肺(fei)炎者該(gai)治療(liao)可挽救生命。如出現支氣管哮喘，采用通常治療(liao)方法.有(you)蠕蟲感(gan)染(ran)的，應使(shi)用驅蟲藥。