嗜(shi)酸(suan)(suan)性(xing)(xing)(xing)細(xi)胞(bao)性(xing)(xing)(xing)肺炎是一(yi)組(zu)病因明確(que)或(huo)尚未明確(que),以嗜(shi)酸(suan)(suan)性(xing)(xing)(xing)細(xi)胞(bao)浸潤為特點(dian),常伴周(zhou)圍血嗜(shi)酸(suan)(suan)細(xi)胞(bao)增多的(de)疾病。有時稱(cheng)為嗜(shi)酸(suan)(suan)性(xing)(xing)(xing)細(xi)胞(bao)增多性(xing)(xing)(xing)肺浸潤(PIE)綜合(he)征。
嗜(shi)酸(suan)性(xing)細(xi)胞性(xing)肺(fei)炎是一組病因明(ming)確或尚未明(ming)確,以嗜(shi)酸(suan)性(xing)細(xi)胞浸潤(run)為特(te)點,常伴(ban)周圍血嗜(shi)酸(suan)細(xi)胞增(zeng)多的疾病.
有時稱為嗜(shi)酸性細胞增多性肺浸潤(PIE)綜合(he)征。
病因包括寄生蟲(如(ru)蛔(hui)(hui)蟲,弓蛔(hui)(hui)蚴蟲和(he)絲蟲),藥物(如(ru)青霉(mei)素,對氨基水楊酸,肼苯噠(da)嗪(qin)(qin),呋喃(nan)妥(tuo)因,氯丙(bing)嗪(qin)(qin),磺胺制劑);化(hua)(hua)學過敏物質(如(ru)蒸氣形式吸(xi)入(ru)的碳化(hua)(hua)鎳(nie));和(he)真菌(如(ru)煙曲(qu)霉(mei),它(ta)引起變應(ying)性(xing)支氣管(guan)肺曲(qu)霉(mei)病見后述).盡管(guan)疑有過敏性(xing)機(ji)制,但大(da)多數(shu)嗜(shi)酸性(xing)細胞(bao)肺炎病因不(bu)明.嗜(shi)酸性(xing)細胞(bao)增多提(ti)示(shi)為第(di)Ⅰ型過敏反應(ying),綜合征的其他特點(血管(guan)炎,圓細胞(bao)浸(jin)潤)提(ti)示(shi)為第(di)Ⅲ型亦可能為第(di)Ⅳ型反應(ying).
嗜(shi)酸(suan)性(xing)細胞性(xing)肺炎(yan)(表76-3)常合(he)并(bing)(bing)有支(zhi)氣管哮喘.合(he)并(bing)(bing)哮喘及病因不明的(de)嗜(shi)酸(suan)性(xing)細胞性(xing)肺炎(yan)可分為(wei)三類:外源性(xing)支(zhi)氣管哮喘伴PIE綜合(he)征(zheng)(zheng),事實上(shang),常為(wei)變(bian)應性(xing)支(zhi)氣管肺曲霉病;內源性(xing)支(zhi)氣管哮喘伴PIE綜合(he)征(zheng)(zheng)(慢性(xing)嗜(shi)酸(suan)性(xing)細胞性(xing)肺炎(yan)),胸片上(shang)常出現特(te)征(zheng)(zheng)性(xing)的(de)肺周圍(wei)部(bu)浸潤(run);和變(bian)應性(xing)肉(rou)芽腫病(Churg-Strauss綜合(he)征(zheng)(zheng),一(yi)種結節(jie)性(xing)多關節(jie)炎(yan)伴肺病變(bian)).單純性(xing)嗜(shi)酸(suan)性(xing)細胞性(xing)肺炎(yan)(LÖffler綜合(he)征(zheng)(zheng))偶可合(he)并(bing)(bing)哮喘.
不(bu)伴哮喘的(de)嗜(shi)(shi)(shi)(shi)酸(suan)(suan)性(xing)(xing)(xing)(xing)細(xi)(xi)(xi)(xi)胞(bao)(bao)(bao)性(xing)(xing)(xing)(xing)肺(fei)炎包括急性(xing)(xing)(xing)(xing)嗜(shi)(shi)(shi)(shi)酸(suan)(suan)性(xing)(xing)(xing)(xing)細(xi)(xi)(xi)(xi)胞(bao)(bao)(bao)性(xing)(xing)(xing)(xing)肺(fei)炎,嗜(shi)(shi)(shi)(shi)酸(suan)(suan)性(xing)(xing)(xing)(xing)細(xi)(xi)(xi)(xi)胞(bao)(bao)(bao)性(xing)(xing)(xing)(xing)肌痛綜(zong)合(he)征(zheng)和嗜(shi)(shi)(shi)(shi)酸(suan)(suan)性(xing)(xing)(xing)(xing)細(xi)(xi)(xi)(xi)胞(bao)(bao)(bao)增(zeng)(zeng)(zeng)多綜(zong)合(he)征(zheng).急性(xing)(xing)(xing)(xing)細(xi)(xi)(xi)(xi)胞(bao)(bao)(bao)性(xing)(xing)(xing)(xing)肺(fei)炎是一種原因不(bu)明的(de)不(bu)同病(bing)種,可(ke)(ke)引起急性(xing)(xing)(xing)(xing)發熱,嚴重(zhong)低(di)氧血(xue)癥,彌(mi)漫性(xing)(xing)(xing)(xing)肺(fei)浸潤以及支氣管肺(fei)泡灌洗(xi)液中的(de)嗜(shi)(shi)(shi)(shi)酸(suan)(suan)性(xing)(xing)(xing)(xing)細(xi)(xi)(xi)(xi)胞(bao)(bao)(bao)比例(li)>25%.經(jing)(jing)皮(pi)質激素治療可(ke)(ke)迅速,徹底緩(huan)解.嗜(shi)(shi)(shi)(shi)酸(suan)(suan)性(xing)(xing)(xing)(xing)細(xi)(xi)(xi)(xi)胞(bao)(bao)(bao)性(xing)(xing)(xing)(xing)肌痛綜(zong)合(he)征(zheng)與食用(yong)大量的(de)作(zuo)為食物(wu)添(tian)加(jia)劑的(de)L-色氨酸(suan)(suan)有關.肺(fei)浸潤偶可(ke)(ke)伴有肌痛,肌無力,皮(pi)疹(zhen)和類似(si)于硬(ying)皮(pi)病(bing)的(de)軟組織硬(ying)結(jie).嗜(shi)(shi)(shi)(shi)酸(suan)(suan)性(xing)(xing)(xing)(xing)細(xi)(xi)(xi)(xi)胞(bao)(bao)(bao)增(zeng)(zeng)(zeng)多綜(zong)合(he)征(zheng)的(de)診(zhen)斷標(biao)準為持(chi)續性(xing)(xing)(xing)(xing)嗜(shi)(shi)(shi)(shi)酸(suan)(suan)性(xing)(xing)(xing)(xing)細(xi)(xi)(xi)(xi)胞(bao)(bao)(bao)增(zeng)(zeng)(zeng)多(>1500/mm3)達6個(ge)月以上,缺乏(fa)其他(ta)引起嗜(shi)(shi)(shi)(shi)酸(suan)(suan)性(xing)(xing)(xing)(xing)細(xi)(xi)(xi)(xi)胞(bao)(bao)(bao)增(zeng)(zeng)(zeng)多的(de)病(bing)因,累(lei)及心(xin),肝,脾,中樞神經(jing)(jing)系(xi)統(tong)或肺(fei),心(xin)臟最常(chang)(chang)受累(lei).發熱,體重(zhong)減輕和貧血(xue)也常(chang)(chang)見,常(chang)(chang)可(ke)(ke)發生動脈而(er)非靜脈性(xing)(xing)(xing)(xing)血(xue)栓(shuan)栓(shuan)塞疾病(bing)。
特(te)征性(xing)改(gai)變包括(kuo)嗜酸(suan)(suan)性(xing)細(xi)(xi)胞(bao),大(da)單核細(xi)(xi)胞(bao)充(chong)滿肺泡(pao),肺泡(pao)間隔內嗜酸(suan)(suan)性(xing)細(xi)(xi)胞(bao),漿(jiang)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(guan)內粘液填塞(sai)和(he)血管(guan)浸潤。
癥(zheng)狀和(he)體征可輕微,也(ye)可危及(ji)生命.可伴低度發(fa)熱,輕度(如(ru)有(you))呼(hu)吸道癥(zheng)狀,可即時恢復(fu).其他類(lei)型的PIE綜合征,可有(you)發(fa)熱和(he)支氣管哮喘癥(zheng)狀,包括咳嗽(sou),喘鳴和(he)靜息時呼(hu)吸困難.如(ru)不治療,慢性(xing)嗜酸性(xing)細胞性(xing)肺(fei)炎常進行性(xing)發(fa)展(zhan)至危及(ji)生命,類(lei)似(si)急性(xing)嗜酸性(xing)肺(fei)炎.常有(you)明顯增高(gao)的嗜酸性(xing)細胞(20%~40%,有(you)時更高(gao)).胸部X線表現(xian)(xian)為(wei)不同(tong)肺(fei)葉內有(you)迅速(su)出現(xian)(xian)及(ji)迅速(su)消失(shi)的浸潤病灶(游走性(xing)浸潤)。
根據病人居住的地理區域尋找(zhao)致病蠕(ru)蟲(chong)(chong),痰內可(ke)找(zhao)到寄(ji)生蟲(chong)(chong)和(he)煙曲霉,需仔細(xi)詢問用(yong)藥史(shi).鑒別診斷包括結(jie)核,結(jie)節病,霍奇金病和(he)其他淋巴組織(zhi)增生性疾(ji)病,肺嗜(shi)酸性細(xi)胞(bao)性肉芽(ya)腫(zhong),脫屑性間質性肺炎(yan)和(he)膠原性血(xue)管疾(ji)病。過敏性肺炎(yan)和(he)Wegener肉芽(ya)腫(zhong)常不伴嗜(shi)酸性細(xi)胞(bao)增多。
本(ben)病可(ke)為自(zi)限性,呈良性,可(ke)不(bu)需治療(liao).如癥狀嚴重,使用皮質類固醇常(chang)有極好效果;對于(yu)急性嗜(shi)酸(suan)性細胞肺炎和(he)特發性慢性嗜(shi)酸(suan)性肺炎者該治療(liao)可(ke)挽救生命。如出現支氣管(guan)哮(xiao)喘,采用通常(chang)治療(liao)方法(fa).有蠕蟲感染的,應使用驅蟲藥。