嗜酸(suan)(suan)性(xing)(xing)細胞(bao)性(xing)(xing)肺炎是(shi)一組病因明確或尚未明確,以嗜酸(suan)(suan)性(xing)(xing)細胞(bao)浸(jin)潤為(wei)特點,常(chang)伴(ban)周圍(wei)血嗜酸(suan)(suan)細胞(bao)增多的疾病。有時(shi)稱(cheng)為(wei)嗜酸(suan)(suan)性(xing)(xing)細胞(bao)增多性(xing)(xing)肺浸(jin)潤(PIE)綜(zong)合征。
嗜(shi)(shi)酸性細(xi)胞性肺炎(yan)是(shi)一(yi)組(zu)病因明確或尚未明確,以嗜(shi)(shi)酸性細(xi)胞浸潤為特(te)點,常伴(ban)周圍血嗜(shi)(shi)酸細(xi)胞增多的疾病.
有(you)時稱為嗜(shi)酸性細(xi)胞(bao)增(zeng)多性肺浸潤(run)(PIE)綜合征。
病(bing)(bing)因包括寄生蟲(如(ru)蛔(hui)蟲,弓蛔(hui)蚴蟲和(he)絲蟲),藥物(如(ru)青霉(mei)素,對氨(an)基水楊(yang)酸,肼苯(ben)噠(da)嗪(qin)(qin),呋(fu)喃妥因,氯丙嗪(qin)(qin),磺胺制(zhi)劑);化學(xue)過(guo)敏(min)物質(如(ru)蒸氣(qi)(qi)形式吸入(ru)的(de)碳(tan)化鎳);和(he)真菌(如(ru)煙(yan)曲霉(mei),它引(yin)起(qi)變應(ying)性支氣(qi)(qi)管肺(fei)曲霉(mei)病(bing)(bing)見后述(shu)).盡管疑(yi)有過(guo)敏(min)性機制(zhi),但(dan)大多數嗜酸性細(xi)(xi)胞肺(fei)炎病(bing)(bing)因不明.嗜酸性細(xi)(xi)胞增(zeng)多提示為(wei)第Ⅰ型(xing)過(guo)敏(min)反應(ying),綜合征的(de)其(qi)他特點(血(xue)管炎,圓細(xi)(xi)胞浸潤)提示為(wei)第Ⅲ型(xing)亦(yi)可能為(wei)第Ⅳ型(xing)反應(ying).
嗜(shi)酸性(xing)細(xi)胞(bao)性(xing)肺炎(表76-3)常(chang)合(he)(he)并有支(zhi)(zhi)氣管(guan)哮(xiao)喘(chuan).合(he)(he)并哮(xiao)喘(chuan)及病(bing)因不(bu)明的嗜(shi)酸性(xing)細(xi)胞(bao)性(xing)肺炎可分(fen)為三類(lei):外源(yuan)性(xing)支(zhi)(zhi)氣管(guan)哮(xiao)喘(chuan)伴PIE綜(zong)合(he)(he)征,事實上,常(chang)為變(bian)(bian)應性(xing)支(zhi)(zhi)氣管(guan)肺曲霉(mei)病(bing);內源(yuan)性(xing)支(zhi)(zhi)氣管(guan)哮(xiao)喘(chuan)伴PIE綜(zong)合(he)(he)征(慢性(xing)嗜(shi)酸性(xing)細(xi)胞(bao)性(xing)肺炎),胸(xiong)片上常(chang)出現特征性(xing)的肺周圍部浸潤(run);和變(bian)(bian)應性(xing)肉芽腫病(bing)(Churg-Strauss綜(zong)合(he)(he)征,一種結節性(xing)多(duo)關(guan)節炎伴肺病(bing)變(bian)(bian)).單(dan)純性(xing)嗜(shi)酸性(xing)細(xi)胞(bao)性(xing)肺炎(LÖffler綜(zong)合(he)(he)征)偶可合(he)(he)并哮(xiao)喘(chuan).
不(bu)伴哮喘的(de)嗜(shi)酸(suan)性(xing)(xing)(xing)(xing)細(xi)(xi)胞(bao)性(xing)(xing)(xing)(xing)肺(fei)(fei)炎包括急(ji)性(xing)(xing)(xing)(xing)嗜(shi)酸(suan)性(xing)(xing)(xing)(xing)細(xi)(xi)胞(bao)性(xing)(xing)(xing)(xing)肺(fei)(fei)炎,嗜(shi)酸(suan)性(xing)(xing)(xing)(xing)細(xi)(xi)胞(bao)性(xing)(xing)(xing)(xing)肌(ji)痛綜(zong)(zong)合(he)征和嗜(shi)酸(suan)性(xing)(xing)(xing)(xing)細(xi)(xi)胞(bao)增多(duo)(duo)綜(zong)(zong)合(he)征.急(ji)性(xing)(xing)(xing)(xing)細(xi)(xi)胞(bao)性(xing)(xing)(xing)(xing)肺(fei)(fei)炎是一種(zhong)原因不(bu)明(ming)的(de)不(bu)同病種(zhong),可引起急(ji)性(xing)(xing)(xing)(xing)發(fa)熱(re),嚴重低氧血癥,彌漫性(xing)(xing)(xing)(xing)肺(fei)(fei)浸(jin)潤以(yi)及支氣管肺(fei)(fei)泡灌洗液中(zhong)的(de)嗜(shi)酸(suan)性(xing)(xing)(xing)(xing)細(xi)(xi)胞(bao)比例>25%.經(jing)皮質激素治療可迅速(su),徹底(di)緩解(jie).嗜(shi)酸(suan)性(xing)(xing)(xing)(xing)細(xi)(xi)胞(bao)性(xing)(xing)(xing)(xing)肌(ji)痛綜(zong)(zong)合(he)征與食(shi)用(yong)大(da)量的(de)作為(wei)食(shi)物添(tian)加劑的(de)L-色氨(an)酸(suan)有(you)(you)關.肺(fei)(fei)浸(jin)潤偶(ou)可伴有(you)(you)肌(ji)痛,肌(ji)無力,皮疹和類似于硬皮病的(de)軟組(zu)織硬結.嗜(shi)酸(suan)性(xing)(xing)(xing)(xing)細(xi)(xi)胞(bao)增多(duo)(duo)綜(zong)(zong)合(he)征的(de)診斷標準為(wei)持續性(xing)(xing)(xing)(xing)嗜(shi)酸(suan)性(xing)(xing)(xing)(xing)細(xi)(xi)胞(bao)增多(duo)(duo)(>1500/mm3)達6個月以(yi)上,缺乏其(qi)他引起嗜(shi)酸(suan)性(xing)(xing)(xing)(xing)細(xi)(xi)胞(bao)增多(duo)(duo)的(de)病因,累及心,肝,脾,中(zhong)樞神經(jing)系統(tong)或肺(fei)(fei),心臟最(zui)常(chang)受累.發(fa)熱(re),體重減(jian)輕(qing)和貧血也常(chang)見,常(chang)可發(fa)生(sheng)動脈而非靜脈性(xing)(xing)(xing)(xing)血栓(shuan)栓(shuan)塞疾病。
特(te)征性改變包(bao)括嗜酸性細(xi)胞,大單(dan)核細(xi)胞充滿(man)肺(fei)泡,肺(fei)泡間隔(ge)內嗜酸性細(xi)胞,漿細(xi)胞和大,小單(dan)核細(xi)胞浸潤(run).也可(ke)有細(xi)支氣(qi)管內粘液(ye)填塞和血管浸潤(run)。
癥狀和體(ti)征可輕微,也可危(wei)及(ji)生(sheng)命.可伴低度發(fa)熱(re),輕度(如有(you))呼(hu)吸道癥狀,可即時(shi)恢復.其他(ta)類型的PIE綜合(he)征,可有(you)發(fa)熱(re)和支氣管哮喘(chuan)(chuan)癥狀,包括(kuo)咳嗽,喘(chuan)(chuan)鳴和靜息時(shi)呼(hu)吸困難.如不治療(liao),慢(man)性(xing)(xing)(xing)嗜(shi)(shi)酸(suan)(suan)(suan)性(xing)(xing)(xing)細胞性(xing)(xing)(xing)肺(fei)炎(yan)常進行性(xing)(xing)(xing)發(fa)展至危(wei)及(ji)生(sheng)命,類似急性(xing)(xing)(xing)嗜(shi)(shi)酸(suan)(suan)(suan)性(xing)(xing)(xing)肺(fei)炎(yan).常有(you)明顯(xian)增(zeng)高的嗜(shi)(shi)酸(suan)(suan)(suan)性(xing)(xing)(xing)細胞(20%~40%,有(you)時(shi)更高).胸部X線(xian)表現為(wei)不同(tong)肺(fei)葉內有(you)迅(xun)(xun)速出現及(ji)迅(xun)(xun)速消失的浸潤病(bing)灶(游(you)走性(xing)(xing)(xing)浸潤)。
根據病(bing)人居住的地理區域尋找致病(bing)蠕(ru)蟲,痰(tan)內可(ke)找到寄生(sheng)蟲和(he)煙曲霉(mei),需仔細詢問(wen)用(yong)藥史.鑒別診(zhen)斷包括結核,結節病(bing),霍奇(qi)金病(bing)和(he)其他淋巴組織增生(sheng)性疾病(bing),肺(fei)嗜酸(suan)性細胞(bao)性肉(rou)芽腫(zhong),脫(tuo)屑性間(jian)質性肺(fei)炎和(he)膠(jiao)原(yuan)性血管疾病(bing)。過敏性肺(fei)炎和(he)Wegener肉(rou)芽腫(zhong)常不伴嗜酸(suan)性細胞(bao)增多。
本(ben)病可為自限性(xing)(xing),呈良性(xing)(xing),可不需治(zhi)療.如癥(zheng)狀(zhuang)嚴重,使用(yong)皮質類固醇常有(you)極好(hao)效果;對(dui)于急性(xing)(xing)嗜酸性(xing)(xing)細胞肺炎(yan)和特(te)發性(xing)(xing)慢性(xing)(xing)嗜酸性(xing)(xing)肺炎(yan)者該治(zhi)療可挽救生(sheng)命。如出(chu)現支氣管哮喘,采(cai)用(yong)通常治(zhi)療方(fang)法.有(you)蠕蟲感染的,應使用(yong)驅蟲藥(yao)。