嗜酸性(xing)細(xi)胞(bao)性(xing)肺(fei)炎是一(yi)組病因明(ming)確(que)或尚未(wei)明(ming)確(que),以嗜酸性(xing)細(xi)胞(bao)浸潤為特點,常伴周圍血嗜酸細(xi)胞(bao)增多的疾病。有時稱(cheng)為嗜酸性(xing)細(xi)胞(bao)增多性(xing)肺(fei)浸潤(PIE)綜合征。
嗜酸(suan)性(xing)細(xi)(xi)胞(bao)性(xing)肺炎是(shi)一組病因明(ming)確(que)或(huo)尚未(wei)明(ming)確(que),以(yi)嗜酸(suan)性(xing)細(xi)(xi)胞(bao)浸潤為特點,常伴(ban)周(zhou)圍血嗜酸(suan)細(xi)(xi)胞(bao)增(zeng)多的疾病.
有時稱為嗜(shi)酸性(xing)細胞增多性(xing)肺浸潤(PIE)綜合征。
病(bing)因(yin)包括寄生蟲(chong)(如蛔蟲(chong),弓蛔蚴蟲(chong)和絲(si)蟲(chong)),藥物(如青霉(mei)(mei)素,對氨基水楊酸,肼(jing)苯噠嗪,呋喃妥因(yin),氯丙嗪,磺(huang)胺制劑);化(hua)學過敏(min)(min)物質(如蒸氣(qi)形式吸入的碳化(hua)鎳(nie));和真菌(如煙(yan)曲霉(mei)(mei),它引起變(bian)應性支氣(qi)管(guan)肺曲霉(mei)(mei)病(bing)見后述(shu)).盡管(guan)疑有過敏(min)(min)性機制,但大多(duo)數嗜酸性細(xi)胞肺炎(yan)(yan)病(bing)因(yin)不明(ming).嗜酸性細(xi)胞增多(duo)提示為第(di)Ⅰ型(xing)過敏(min)(min)反(fan)應,綜(zong)合征的其他(ta)特點(血管(guan)炎(yan)(yan),圓細(xi)胞浸潤)提示為第(di)Ⅲ型(xing)亦可能為第(di)Ⅳ型(xing)反(fan)應.
嗜酸(suan)性(xing)(xing)細(xi)胞(bao)性(xing)(xing)肺(fei)(fei)(fei)(fei)炎(yan)(表76-3)常(chang)合(he)并有支(zhi)(zhi)(zhi)氣管哮喘(chuan)(chuan).合(he)并哮喘(chuan)(chuan)及(ji)病因(yin)不明的嗜酸(suan)性(xing)(xing)細(xi)胞(bao)性(xing)(xing)肺(fei)(fei)(fei)(fei)炎(yan)可(ke)分為三類(lei):外源(yuan)性(xing)(xing)支(zhi)(zhi)(zhi)氣管哮喘(chuan)(chuan)伴(ban)(ban)PIE綜(zong)(zong)合(he)征(zheng),事實上(shang),常(chang)為變應性(xing)(xing)支(zhi)(zhi)(zhi)氣管肺(fei)(fei)(fei)(fei)曲霉病;內源(yuan)性(xing)(xing)支(zhi)(zhi)(zhi)氣管哮喘(chuan)(chuan)伴(ban)(ban)PIE綜(zong)(zong)合(he)征(zheng)(慢性(xing)(xing)嗜酸(suan)性(xing)(xing)細(xi)胞(bao)性(xing)(xing)肺(fei)(fei)(fei)(fei)炎(yan)),胸片上(shang)常(chang)出現特征(zheng)性(xing)(xing)的肺(fei)(fei)(fei)(fei)周圍部浸潤(run);和(he)變應性(xing)(xing)肉(rou)芽腫病(Churg-Strauss綜(zong)(zong)合(he)征(zheng),一種結節性(xing)(xing)多關(guan)節炎(yan)伴(ban)(ban)肺(fei)(fei)(fei)(fei)病變).單純性(xing)(xing)嗜酸(suan)性(xing)(xing)細(xi)胞(bao)性(xing)(xing)肺(fei)(fei)(fei)(fei)炎(yan)(LÖffler綜(zong)(zong)合(he)征(zheng))偶可(ke)合(he)并哮喘(chuan)(chuan).
不(bu)伴(ban)哮喘(chuan)的(de)(de)(de)嗜(shi)(shi)(shi)酸(suan)(suan)(suan)性(xing)(xing)(xing)(xing)(xing)(xing)(xing)(xing)(xing)細(xi)胞(bao)(bao)性(xing)(xing)(xing)(xing)(xing)(xing)(xing)(xing)(xing)肺(fei)(fei)炎包(bao)括急(ji)(ji)性(xing)(xing)(xing)(xing)(xing)(xing)(xing)(xing)(xing)嗜(shi)(shi)(shi)酸(suan)(suan)(suan)性(xing)(xing)(xing)(xing)(xing)(xing)(xing)(xing)(xing)細(xi)胞(bao)(bao)性(xing)(xing)(xing)(xing)(xing)(xing)(xing)(xing)(xing)肺(fei)(fei)炎,嗜(shi)(shi)(shi)酸(suan)(suan)(suan)性(xing)(xing)(xing)(xing)(xing)(xing)(xing)(xing)(xing)細(xi)胞(bao)(bao)性(xing)(xing)(xing)(xing)(xing)(xing)(xing)(xing)(xing)肌痛(tong)綜(zong)合(he)征(zheng)和嗜(shi)(shi)(shi)酸(suan)(suan)(suan)性(xing)(xing)(xing)(xing)(xing)(xing)(xing)(xing)(xing)細(xi)胞(bao)(bao)增(zeng)多(duo)綜(zong)合(he)征(zheng).急(ji)(ji)性(xing)(xing)(xing)(xing)(xing)(xing)(xing)(xing)(xing)細(xi)胞(bao)(bao)性(xing)(xing)(xing)(xing)(xing)(xing)(xing)(xing)(xing)肺(fei)(fei)炎是一種原因不(bu)明的(de)(de)(de)不(bu)同病種,可(ke)引(yin)起急(ji)(ji)性(xing)(xing)(xing)(xing)(xing)(xing)(xing)(xing)(xing)發熱(re),嚴重(zhong)(zhong)低氧血癥,彌漫性(xing)(xing)(xing)(xing)(xing)(xing)(xing)(xing)(xing)肺(fei)(fei)浸潤(run)以及支氣管肺(fei)(fei)泡灌洗液中的(de)(de)(de)嗜(shi)(shi)(shi)酸(suan)(suan)(suan)性(xing)(xing)(xing)(xing)(xing)(xing)(xing)(xing)(xing)細(xi)胞(bao)(bao)比例>25%.經(jing)(jing)皮(pi)質激素治(zhi)療可(ke)迅速,徹底緩(huan)解.嗜(shi)(shi)(shi)酸(suan)(suan)(suan)性(xing)(xing)(xing)(xing)(xing)(xing)(xing)(xing)(xing)細(xi)胞(bao)(bao)性(xing)(xing)(xing)(xing)(xing)(xing)(xing)(xing)(xing)肌痛(tong)綜(zong)合(he)征(zheng)與食用(yong)大(da)量的(de)(de)(de)作(zuo)為食物添加劑(ji)的(de)(de)(de)L-色氨酸(suan)(suan)(suan)有關.肺(fei)(fei)浸潤(run)偶可(ke)伴(ban)有肌痛(tong),肌無(wu)力,皮(pi)疹和類(lei)似于硬皮(pi)病的(de)(de)(de)軟(ruan)組織硬結(jie).嗜(shi)(shi)(shi)酸(suan)(suan)(suan)性(xing)(xing)(xing)(xing)(xing)(xing)(xing)(xing)(xing)細(xi)胞(bao)(bao)增(zeng)多(duo)綜(zong)合(he)征(zheng)的(de)(de)(de)診(zhen)斷標(biao)準為持續(xu)性(xing)(xing)(xing)(xing)(xing)(xing)(xing)(xing)(xing)嗜(shi)(shi)(shi)酸(suan)(suan)(suan)性(xing)(xing)(xing)(xing)(xing)(xing)(xing)(xing)(xing)細(xi)胞(bao)(bao)增(zeng)多(duo)(>1500/mm3)達6個月以上,缺乏其(qi)他引(yin)起嗜(shi)(shi)(shi)酸(suan)(suan)(suan)性(xing)(xing)(xing)(xing)(xing)(xing)(xing)(xing)(xing)細(xi)胞(bao)(bao)增(zeng)多(duo)的(de)(de)(de)病因,累及心,肝,脾,中樞神(shen)經(jing)(jing)系(xi)統(tong)或肺(fei)(fei),心臟最常受累.發熱(re),體重(zhong)(zhong)減輕和貧血也常見,常可(ke)發生動脈(mo)而非(fei)靜脈(mo)性(xing)(xing)(xing)(xing)(xing)(xing)(xing)(xing)(xing)血栓栓塞疾病。
特征性改變包括嗜酸(suan)性細(xi)胞(bao)(bao),大單核細(xi)胞(bao)(bao)充滿肺泡(pao),肺泡(pao)間隔內(nei)嗜酸(suan)性細(xi)胞(bao)(bao),漿細(xi)胞(bao)(bao)和大,小(xiao)單核細(xi)胞(bao)(bao)浸潤.也可有(you)細(xi)支氣(qi)管(guan)(guan)內(nei)粘液填(tian)塞(sai)和血(xue)管(guan)(guan)浸潤。
癥(zheng)狀(zhuang)和體(ti)征可(ke)輕微(wei),也(ye)可(ke)危及生命.可(ke)伴低度(du)發熱,輕度(du)(如(ru)有)呼(hu)吸道癥(zheng)狀(zhuang),可(ke)即時恢復.其(qi)他(ta)類(lei)型的PIE綜合征,可(ke)有發熱和支氣管哮喘癥(zheng)狀(zhuang),包(bao)括咳嗽,喘鳴和靜息時呼(hu)吸困難(nan).如(ru)不治(zhi)療,慢性(xing)(xing)嗜(shi)(shi)酸性(xing)(xing)細胞性(xing)(xing)肺(fei)炎(yan)常(chang)進(jin)行性(xing)(xing)發展至危及生命,類(lei)似急性(xing)(xing)嗜(shi)(shi)酸性(xing)(xing)肺(fei)炎(yan).常(chang)有明顯(xian)增(zeng)高的嗜(shi)(shi)酸性(xing)(xing)細胞(20%~40%,有時更高).胸(xiong)部X線(xian)表(biao)現為不同肺(fei)葉內有迅速出現及迅速消失的浸(jin)潤病灶(zao)(游(you)走(zou)性(xing)(xing)浸(jin)潤)。
根據(ju)病(bing)人(ren)居住的地理區域尋找致病(bing)蠕(ru)蟲,痰內可找到寄生蟲和煙曲霉,需仔細詢問(wen)用藥史(shi).鑒別診斷(duan)包括結(jie)(jie)核,結(jie)(jie)節病(bing),霍奇金病(bing)和其(qi)他淋巴組織增生性(xing)(xing)疾病(bing),肺(fei)嗜酸(suan)性(xing)(xing)細胞(bao)性(xing)(xing)肉(rou)芽(ya)腫(zhong),脫屑性(xing)(xing)間質性(xing)(xing)肺(fei)炎和膠原性(xing)(xing)血管疾病(bing)。過敏性(xing)(xing)肺(fei)炎和Wegener肉(rou)芽(ya)腫(zhong)常不伴嗜酸(suan)性(xing)(xing)細胞(bao)增多(duo)。
本病可(ke)為自(zi)限性(xing),呈良性(xing),可(ke)不需治療(liao).如癥狀嚴重,使(shi)用(yong)(yong)皮質類固醇常(chang)(chang)有(you)極好效果;對于急性(xing)嗜酸性(xing)細胞(bao)肺炎(yan)和(he)特發(fa)性(xing)慢性(xing)嗜酸性(xing)肺炎(yan)者(zhe)該治療(liao)可(ke)挽救生命(ming)。如出(chu)現(xian)支氣管哮喘,采用(yong)(yong)通常(chang)(chang)治療(liao)方法(fa).有(you)蠕蟲感染(ran)的,應(ying)使(shi)用(yong)(yong)驅(qu)蟲藥。