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嗜(shi)酸性細(xi)胞(bao)性肺炎是一(yi)組(zu)病因明確或尚未明確，以嗜(shi)酸性細(xi)胞(bao)浸潤(run)為特點(dian)，常(chang)伴周(zhou)圍(wei)血(xue)嗜(shi)酸細(xi)胞(bao)增多(duo)的疾病。有(you)時(shi)稱為嗜(shi)酸性細(xi)胞(bao)增多(duo)性肺浸潤(run)(PIE)綜合征。

基本概述

嗜酸性(xing)細胞(bao)性(xing)肺炎是一組病因明確(que)或尚未明確(que)，以(yi)嗜酸性(xing)細胞(bao)浸潤為特點(dian)，常伴周圍血嗜酸細胞(bao)增多的(de)疾(ji)病.

有時稱(cheng)為嗜(shi)酸性細胞(bao)增多(duo)性肺浸(jin)潤(run)（PIE）綜合征。

病因學和發病機制

病因(yin)包括寄生蟲(chong)（如(ru)蛔蟲(chong)，弓蛔蚴蟲(chong)和絲(si)蟲(chong)），藥物（如(ru)青霉素，對氨基水楊酸，肼苯噠嗪，呋喃妥因(yin)，氯(lv)丙嗪，磺胺制劑）；化學(xue)過(guo)敏(min)物質（如(ru)蒸氣(qi)形式吸入的(de)(de)碳化鎳）；和真(zhen)菌（如(ru)煙曲霉，它引起變應性支(zhi)氣(qi)管(guan)(guan)肺(fei)曲霉病見后述(shu)）.盡管(guan)(guan)疑有過(guo)敏(min)性機制，但大多(duo)數(shu)嗜酸性細胞肺(fei)炎(yan)病因(yin)不(bu)明.嗜酸性細胞增多(duo)提(ti)(ti)示為(wei)第Ⅰ型(xing)過(guo)敏(min)反(fan)應，綜合征的(de)(de)其他(ta)特點（血管(guan)(guan)炎(yan)，圓細胞浸潤）提(ti)(ti)示為(wei)第Ⅲ型(xing)亦可能為(wei)第Ⅳ型(xing)反(fan)應.

嗜酸性(xing)(xing)(xing)(xing)(xing)細(xi)胞性(xing)(xing)(xing)(xing)(xing)肺(fei)(fei)(fei)炎（表(biao)76-3）常合(he)并有(you)支(zhi)氣(qi)管(guan)哮(xiao)(xiao)喘(chuan)(chuan).合(he)并哮(xiao)(xiao)喘(chuan)(chuan)及(ji)病(bing)(bing)因不(bu)明的(de)嗜酸性(xing)(xing)(xing)(xing)(xing)細(xi)胞性(xing)(xing)(xing)(xing)(xing)肺(fei)(fei)(fei)炎可分為三(san)類(lei)：外源性(xing)(xing)(xing)(xing)(xing)支(zhi)氣(qi)管(guan)哮(xiao)(xiao)喘(chuan)(chuan)伴PIE綜(zong)合(he)征(zheng)，事(shi)實上，常為變應性(xing)(xing)(xing)(xing)(xing)支(zhi)氣(qi)管(guan)肺(fei)(fei)(fei)曲霉(mei)病(bing)(bing)；內源性(xing)(xing)(xing)(xing)(xing)支(zhi)氣(qi)管(guan)哮(xiao)(xiao)喘(chuan)(chuan)伴PIE綜(zong)合(he)征(zheng)（慢性(xing)(xing)(xing)(xing)(xing)嗜酸性(xing)(xing)(xing)(xing)(xing)細(xi)胞性(xing)(xing)(xing)(xing)(xing)肺(fei)(fei)(fei)炎），胸片上常出(chu)現(xian)特(te)征(zheng)性(xing)(xing)(xing)(xing)(xing)的(de)肺(fei)(fei)(fei)周圍部(bu)浸(jin)潤；和變應性(xing)(xing)(xing)(xing)(xing)肉(rou)芽腫病(bing)(bing)（Churg-Strauss綜(zong)合(he)征(zheng)，一種結節(jie)性(xing)(xing)(xing)(xing)(xing)多關節(jie)炎伴肺(fei)(fei)(fei)病(bing)(bing)變）.單(dan)純(chun)性(xing)(xing)(xing)(xing)(xing)嗜酸性(xing)(xing)(xing)(xing)(xing)細(xi)胞性(xing)(xing)(xing)(xing)(xing)肺(fei)(fei)(fei)炎（LÖffler綜(zong)合(he)征(zheng)）偶可合(he)并哮(xiao)(xiao)喘(chuan)(chuan).

不伴(ban)哮喘的嗜(shi)酸(suan)(suan)(suan)性(xing)(xing)(xing)(xing)(xing)細(xi)(xi)(xi)胞(bao)(bao)(bao)性(xing)(xing)(xing)(xing)(xing)肺(fei)(fei)(fei)炎包括急(ji)性(xing)(xing)(xing)(xing)(xing)嗜(shi)酸(suan)(suan)(suan)性(xing)(xing)(xing)(xing)(xing)細(xi)(xi)(xi)胞(bao)(bao)(bao)性(xing)(xing)(xing)(xing)(xing)肺(fei)(fei)(fei)炎，嗜(shi)酸(suan)(suan)(suan)性(xing)(xing)(xing)(xing)(xing)細(xi)(xi)(xi)胞(bao)(bao)(bao)性(xing)(xing)(xing)(xing)(xing)肌痛(tong)綜(zong)合征和(he)嗜(shi)酸(suan)(suan)(suan)性(xing)(xing)(xing)(xing)(xing)細(xi)(xi)(xi)胞(bao)(bao)(bao)增(zeng)多(duo)綜(zong)合征.急(ji)性(xing)(xing)(xing)(xing)(xing)細(xi)(xi)(xi)胞(bao)(bao)(bao)性(xing)(xing)(xing)(xing)(xing)肺(fei)(fei)(fei)炎是一種原因不明的不同病(bing)(bing)種，可(ke)(ke)引起(qi)(qi)急(ji)性(xing)(xing)(xing)(xing)(xing)發熱，嚴重(zhong)低氧血(xue)癥，彌(mi)漫性(xing)(xing)(xing)(xing)(xing)肺(fei)(fei)(fei)浸潤(run)以及(ji)支氣管肺(fei)(fei)(fei)泡(pao)灌洗液中的嗜(shi)酸(suan)(suan)(suan)性(xing)(xing)(xing)(xing)(xing)細(xi)(xi)(xi)胞(bao)(bao)(bao)比例>25%.經皮質激素治療可(ke)(ke)迅速，徹底緩解(jie).嗜(shi)酸(suan)(suan)(suan)性(xing)(xing)(xing)(xing)(xing)細(xi)(xi)(xi)胞(bao)(bao)(bao)性(xing)(xing)(xing)(xing)(xing)肌痛(tong)綜(zong)合征與食用大量的作為食物(wu)添加劑的L-色氨酸(suan)(suan)(suan)有關.肺(fei)(fei)(fei)浸潤(run)偶可(ke)(ke)伴(ban)有肌痛(tong)，肌無力，皮疹和(he)類似于硬皮病(bing)(bing)的軟(ruan)組織硬結(jie).嗜(shi)酸(suan)(suan)(suan)性(xing)(xing)(xing)(xing)(xing)細(xi)(xi)(xi)胞(bao)(bao)(bao)增(zeng)多(duo)綜(zong)合征的診斷標準為持(chi)續性(xing)(xing)(xing)(xing)(xing)嗜(shi)酸(suan)(suan)(suan)性(xing)(xing)(xing)(xing)(xing)細(xi)(xi)(xi)胞(bao)(bao)(bao)增(zeng)多(duo)（>1500/mm3）達6個月以上，缺乏其他引起(qi)(qi)嗜(shi)酸(suan)(suan)(suan)性(xing)(xing)(xing)(xing)(xing)細(xi)(xi)(xi)胞(bao)(bao)(bao)增(zeng)多(duo)的病(bing)(bing)因，累(lei)及(ji)心，肝，脾(pi)，中樞神經系統或(huo)肺(fei)(fei)(fei)，心臟最(zui)常(chang)受累(lei).發熱，體重(zhong)減輕和(he)貧血(xue)也(ye)常(chang)見,常(chang)可(ke)(ke)發生動脈而非靜脈性(xing)(xing)(xing)(xing)(xing)血(xue)栓(shuan)栓(shuan)塞疾(ji)病(bing)(bing)。

特(te)征性(xing)(xing)改(gai)變包括嗜酸(suan)性(xing)(xing)細(xi)胞(bao)，大(da)單核細(xi)胞(bao)充(chong)滿肺(fei)泡(pao)，肺(fei)泡(pao)間(jian)隔內嗜酸(suan)性(xing)(xing)細(xi)胞(bao)，漿細(xi)胞(bao)和大(da)，小單核細(xi)胞(bao)浸潤.也可有細(xi)支氣管(guan)內粘液填塞和血管(guan)浸潤。

癥狀和體征

癥狀(zhuang)(zhuang)(zhuang)和體征(zheng)(zheng)可(ke)(ke)輕(qing)微，也可(ke)(ke)危及(ji)生命(ming).可(ke)(ke)伴(ban)低度發熱，輕(qing)度（如有(you)(you)）呼(hu)吸道癥狀(zhuang)(zhuang)(zhuang)，可(ke)(ke)即時恢復.其他類(lei)(lei)型的PIE綜合征(zheng)(zheng)，可(ke)(ke)有(you)(you)發熱和支氣管(guan)哮喘(chuan)癥狀(zhuang)(zhuang)(zhuang)，包括咳嗽，喘(chuan)鳴和靜息時呼(hu)吸困(kun)難(nan).如不治療，慢性(xing)嗜(shi)酸性(xing)細胞性(xing)肺(fei)炎常進行性(xing)發展至危及(ji)生命(ming)，類(lei)(lei)似(si)急性(xing)嗜(shi)酸性(xing)肺(fei)炎.常有(you)(you)明顯(xian)增高的嗜(shi)酸性(xing)細胞（20%~40%，有(you)(you)時更高）.胸(xiong)部X線(xian)表現為不同肺(fei)葉內有(you)(you)迅速(su)出現及(ji)迅速(su)消(xiao)失的浸潤病灶（游(you)走性(xing)浸潤）。

臨床診斷

根(gen)據病(bing)人居住的(de)地理區域尋(xun)找(zhao)致病(bing)蠕蟲，痰內可找(zhao)到寄生蟲和煙曲(qu)霉，需仔細詢問用藥(yao)史(shi).鑒別(bie)診斷(duan)包(bao)括結核，結節病(bing)，霍奇金病(bing)和其(qi)他淋巴組織增生性疾病(bing)，肺嗜酸性細胞性肉(rou)芽腫，脫(tuo)屑(xie)性間質性肺炎(yan)和膠原(yuan)性血管疾病(bing)。過敏性肺炎(yan)和Wegener肉(rou)芽腫常不(bu)伴嗜酸性細胞增多。

治療

本病可(ke)(ke)為自限(xian)性(xing)，呈(cheng)良性(xing)，可(ke)(ke)不需(xu)治(zhi)療.如癥(zheng)狀嚴重，使用皮(pi)質(zhi)類(lei)固醇常有極(ji)好效(xiao)果；對于急性(xing)嗜(shi)酸性(xing)細(xi)胞(bao)肺(fei)炎(yan)(yan)和特發性(xing)慢(man)性(xing)嗜(shi)酸性(xing)肺(fei)炎(yan)(yan)者該治(zhi)療可(ke)(ke)挽救(jiu)生命。如出現(xian)支氣管哮(xiao)喘(chuan)，采用通常治(zhi)療方法.有蠕蟲感染的(de)，應使用驅(qu)蟲藥。