嗜酸(suan)性(xing)(xing)細胞性(xing)(xing)肺(fei)炎是一組(zu)病因明確(que)或尚未明確(que),以嗜酸(suan)性(xing)(xing)細胞浸(jin)潤為特點,常伴(ban)周圍血嗜酸(suan)細胞增(zeng)多的疾病。有(you)時(shi)稱(cheng)為嗜酸(suan)性(xing)(xing)細胞增(zeng)多性(xing)(xing)肺(fei)浸(jin)潤(PIE)綜(zong)合征(zheng)。
嗜(shi)酸性細(xi)胞性肺炎是(shi)一組病因明確或尚未明確,以嗜(shi)酸性細(xi)胞浸潤為特點,常伴周(zhou)圍血嗜(shi)酸細(xi)胞增多的(de)疾病.
有時稱為嗜(shi)酸性細胞增多性肺浸(jin)潤(PIE)綜合征(zheng)。
病(bing)(bing)因包括(kuo)寄(ji)生(sheng)蟲(如(ru)蛔蟲,弓蛔蚴(you)蟲和絲蟲),藥物(如(ru)青霉素,對氨基水楊酸(suan),肼苯噠嗪(qin),呋喃妥因,氯丙嗪(qin),磺胺制(zhi)劑);化(hua)學過(guo)敏(min)物質(如(ru)蒸氣形式吸入的(de)碳化(hua)鎳(nie));和真菌(jun)(如(ru)煙曲霉,它引起變應性(xing)(xing)支(zhi)氣管肺曲霉病(bing)(bing)見后述).盡管疑有過(guo)敏(min)性(xing)(xing)機制(zhi),但(dan)大多(duo)數嗜酸(suan)性(xing)(xing)細胞肺炎(yan)病(bing)(bing)因不明.嗜酸(suan)性(xing)(xing)細胞增多(duo)提示(shi)為(wei)第(di)(di)Ⅰ型(xing)過(guo)敏(min)反應,綜(zong)合征的(de)其他特點(dian)(血管炎(yan),圓細胞浸潤)提示(shi)為(wei)第(di)(di)Ⅲ型(xing)亦可能為(wei)第(di)(di)Ⅳ型(xing)反應.
嗜酸性(xing)(xing)(xing)(xing)細(xi)(xi)胞(bao)(bao)性(xing)(xing)(xing)(xing)肺炎(yan)(表76-3)常(chang)合(he)并有支氣管哮(xiao)喘.合(he)并哮(xiao)喘及病(bing)因不(bu)明的(de)嗜酸性(xing)(xing)(xing)(xing)細(xi)(xi)胞(bao)(bao)性(xing)(xing)(xing)(xing)肺炎(yan)可(ke)分為(wei)三類(lei):外源(yuan)性(xing)(xing)(xing)(xing)支氣管哮(xiao)喘伴(ban)PIE綜(zong)合(he)征,事(shi)實(shi)上,常(chang)為(wei)變(bian)(bian)應(ying)性(xing)(xing)(xing)(xing)支氣管肺曲(qu)霉病(bing);內源(yuan)性(xing)(xing)(xing)(xing)支氣管哮(xiao)喘伴(ban)PIE綜(zong)合(he)征(慢性(xing)(xing)(xing)(xing)嗜酸性(xing)(xing)(xing)(xing)細(xi)(xi)胞(bao)(bao)性(xing)(xing)(xing)(xing)肺炎(yan)),胸(xiong)片上常(chang)出現特(te)征性(xing)(xing)(xing)(xing)的(de)肺周(zhou)圍(wei)部浸潤(run);和變(bian)(bian)應(ying)性(xing)(xing)(xing)(xing)肉芽(ya)腫病(bing)(Churg-Strauss綜(zong)合(he)征,一種結節(jie)性(xing)(xing)(xing)(xing)多關節(jie)炎(yan)伴(ban)肺病(bing)變(bian)(bian)).單純性(xing)(xing)(xing)(xing)嗜酸性(xing)(xing)(xing)(xing)細(xi)(xi)胞(bao)(bao)性(xing)(xing)(xing)(xing)肺炎(yan)(LÖffler綜(zong)合(he)征)偶可(ke)合(he)并哮(xiao)喘.
不(bu)(bu)伴哮喘的嗜(shi)(shi)(shi)(shi)酸(suan)性(xing)(xing)(xing)(xing)(xing)(xing)細(xi)(xi)胞(bao)(bao)(bao)(bao)性(xing)(xing)(xing)(xing)(xing)(xing)肺(fei)炎包括急性(xing)(xing)(xing)(xing)(xing)(xing)嗜(shi)(shi)(shi)(shi)酸(suan)性(xing)(xing)(xing)(xing)(xing)(xing)細(xi)(xi)胞(bao)(bao)(bao)(bao)性(xing)(xing)(xing)(xing)(xing)(xing)肺(fei)炎,嗜(shi)(shi)(shi)(shi)酸(suan)性(xing)(xing)(xing)(xing)(xing)(xing)細(xi)(xi)胞(bao)(bao)(bao)(bao)性(xing)(xing)(xing)(xing)(xing)(xing)肌痛綜合(he)(he)征和嗜(shi)(shi)(shi)(shi)酸(suan)性(xing)(xing)(xing)(xing)(xing)(xing)細(xi)(xi)胞(bao)(bao)(bao)(bao)增多(duo)(duo)綜合(he)(he)征.急性(xing)(xing)(xing)(xing)(xing)(xing)細(xi)(xi)胞(bao)(bao)(bao)(bao)性(xing)(xing)(xing)(xing)(xing)(xing)肺(fei)炎是一種原(yuan)因不(bu)(bu)明的不(bu)(bu)同病(bing)種,可(ke)(ke)引起急性(xing)(xing)(xing)(xing)(xing)(xing)發熱,嚴(yan)重低氧血癥,彌(mi)漫(man)性(xing)(xing)(xing)(xing)(xing)(xing)肺(fei)浸(jin)潤以(yi)及支氣管(guan)肺(fei)泡灌(guan)洗液中的嗜(shi)(shi)(shi)(shi)酸(suan)性(xing)(xing)(xing)(xing)(xing)(xing)細(xi)(xi)胞(bao)(bao)(bao)(bao)比例>25%.經皮(pi)質激素(su)治療可(ke)(ke)迅速,徹底緩(huan)解(jie).嗜(shi)(shi)(shi)(shi)酸(suan)性(xing)(xing)(xing)(xing)(xing)(xing)細(xi)(xi)胞(bao)(bao)(bao)(bao)性(xing)(xing)(xing)(xing)(xing)(xing)肌痛綜合(he)(he)征與食用大量(liang)的作(zuo)為(wei)(wei)食物添加劑的L-色氨酸(suan)有關.肺(fei)浸(jin)潤偶可(ke)(ke)伴有肌痛,肌無力,皮(pi)疹和類似于硬皮(pi)病(bing)的軟組織硬結.嗜(shi)(shi)(shi)(shi)酸(suan)性(xing)(xing)(xing)(xing)(xing)(xing)細(xi)(xi)胞(bao)(bao)(bao)(bao)增多(duo)(duo)綜合(he)(he)征的診(zhen)斷標(biao)準為(wei)(wei)持續(xu)性(xing)(xing)(xing)(xing)(xing)(xing)嗜(shi)(shi)(shi)(shi)酸(suan)性(xing)(xing)(xing)(xing)(xing)(xing)細(xi)(xi)胞(bao)(bao)(bao)(bao)增多(duo)(duo)(>1500/mm3)達6個月以(yi)上(shang),缺(que)乏(fa)其他引起嗜(shi)(shi)(shi)(shi)酸(suan)性(xing)(xing)(xing)(xing)(xing)(xing)細(xi)(xi)胞(bao)(bao)(bao)(bao)增多(duo)(duo)的病(bing)因,累及心,肝,脾,中樞(shu)神經系(xi)統或肺(fei),心臟最(zui)常受累.發熱,體重減(jian)輕和貧血也(ye)常見,常可(ke)(ke)發生(sheng)動脈(mo)(mo)而非靜(jing)脈(mo)(mo)性(xing)(xing)(xing)(xing)(xing)(xing)血栓栓塞(sai)疾病(bing)。
特征性改變包括嗜(shi)酸性細(xi)胞(bao),大(da)單(dan)核細(xi)胞(bao)充滿肺泡(pao),肺泡(pao)間隔(ge)內(nei)嗜(shi)酸性細(xi)胞(bao),漿細(xi)胞(bao)和(he)大(da),小單(dan)核細(xi)胞(bao)浸潤(run).也可有細(xi)支氣(qi)管(guan)內(nei)粘液(ye)填塞和(he)血管(guan)浸潤(run)。
癥(zheng)(zheng)狀和體征可(ke)(ke)輕(qing)微,也(ye)可(ke)(ke)危(wei)及生命.可(ke)(ke)伴低度(du)發熱,輕(qing)度(du)(如有)呼吸道(dao)癥(zheng)(zheng)狀,可(ke)(ke)即(ji)時恢復.其(qi)他類型的(de)PIE綜合征,可(ke)(ke)有發熱和支氣管哮喘(chuan)癥(zheng)(zheng)狀,包括(kuo)咳嗽,喘(chuan)鳴和靜息(xi)時呼吸困難.如不(bu)治(zhi)療,慢性(xing)(xing)嗜酸性(xing)(xing)細胞(bao)(bao)性(xing)(xing)肺(fei)炎(yan)常進(jin)行性(xing)(xing)發展(zhan)至危(wei)及生命,類似急性(xing)(xing)嗜酸性(xing)(xing)肺(fei)炎(yan).常有明顯增高的(de)嗜酸性(xing)(xing)細胞(bao)(bao)(20%~40%,有時更(geng)高).胸部X線表現(xian)為不(bu)同肺(fei)葉內有迅(xun)速出現(xian)及迅(xun)速消失的(de)浸潤(run)病灶(游走性(xing)(xing)浸潤(run))。
根據病人居住的地理區域尋(xun)找(zhao)致病蠕蟲,痰內(nei)可找(zhao)到寄生(sheng)蟲和(he)煙曲霉,需仔細詢問(wen)用(yong)藥史.鑒別(bie)診斷包括(kuo)結核,結節病,霍奇金病和(he)其他淋巴組織增(zeng)生(sheng)性(xing)疾病,肺(fei)嗜酸性(xing)細胞(bao)性(xing)肉芽腫(zhong),脫屑性(xing)間質性(xing)肺(fei)炎和(he)膠原性(xing)血管疾病。過敏性(xing)肺(fei)炎和(he)Wegener肉芽腫(zhong)常不伴嗜酸性(xing)細胞(bao)增(zeng)多。
本病(bing)可(ke)為自限性,呈良性,可(ke)不需治(zhi)療(liao).如癥狀嚴(yan)重,使(shi)用(yong)皮質類固醇常有(you)極好(hao)效果;對于急性嗜(shi)酸性細(xi)胞肺(fei)炎(yan)和(he)特發(fa)性慢性嗜(shi)酸性肺(fei)炎(yan)者該(gai)治(zhi)療(liao)可(ke)挽(wan)救生命。如出現支氣管哮喘,采用(yong)通常治(zhi)療(liao)方法.有(you)蠕蟲感(gan)染的,應使(shi)用(yong)驅(qu)蟲藥。