嗜(shi)酸(suan)(suan)(suan)性(xing)細(xi)胞(bao)性(xing)肺(fei)炎(yan)是一組(zu)病因明確或尚未明確,以嗜(shi)酸(suan)(suan)(suan)性(xing)細(xi)胞(bao)浸(jin)潤為(wei)特(te)點(dian),常伴周圍血嗜(shi)酸(suan)(suan)(suan)細(xi)胞(bao)增多(duo)的疾(ji)病。有(you)時稱(cheng)為(wei)嗜(shi)酸(suan)(suan)(suan)性(xing)細(xi)胞(bao)增多(duo)性(xing)肺(fei)浸(jin)潤(PIE)綜合征。
嗜酸(suan)性細(xi)胞性肺炎是一組病(bing)因明確(que)或尚(shang)未明確(que),以嗜酸(suan)性細(xi)胞浸潤為特點,常伴周圍血嗜酸(suan)細(xi)胞增多的疾(ji)病(bing).
有時(shi)稱為嗜酸性(xing)細胞增多性(xing)肺(fei)浸潤(PIE)綜合(he)征。
病(bing)(bing)因包括(kuo)寄生(sheng)蟲(如(ru)蛔蟲,弓(gong)蛔蚴蟲和絲蟲),藥(yao)物(wu)(如(ru)青霉(mei)(mei)(mei)素(su),對(dui)氨基(ji)水楊酸,肼苯噠嗪(qin),呋喃妥因,氯丙嗪(qin),磺胺制劑);化(hua)學過(guo)敏(min)物(wu)質(如(ru)蒸(zheng)氣形式吸入的碳化(hua)鎳);和真菌(如(ru)煙曲霉(mei)(mei)(mei),它引起(qi)變應性(xing)支氣管肺曲霉(mei)(mei)(mei)病(bing)(bing)見后述).盡管疑有過(guo)敏(min)性(xing)機制,但大多(duo)數嗜酸性(xing)細(xi)胞肺炎(yan)病(bing)(bing)因不明(ming).嗜酸性(xing)細(xi)胞增多(duo)提示為第(di)Ⅰ型過(guo)敏(min)反應,綜合征的其他特點(血(xue)管炎(yan),圓細(xi)胞浸(jin)潤)提示為第(di)Ⅲ型亦可能為第(di)Ⅳ型反應.
嗜酸性(xing)細(xi)(xi)胞(bao)性(xing)肺(fei)炎(yan)(表76-3)常(chang)合(he)并有支(zhi)氣(qi)管哮喘.合(he)并哮喘及病因不明的嗜酸性(xing)細(xi)(xi)胞(bao)性(xing)肺(fei)炎(yan)可分為三類(lei):外源性(xing)支(zhi)氣(qi)管哮喘伴(ban)PIE綜(zong)(zong)合(he)征(zheng),事實(shi)上(shang),常(chang)為變應(ying)性(xing)支(zhi)氣(qi)管肺(fei)曲霉病;內源性(xing)支(zhi)氣(qi)管哮喘伴(ban)PIE綜(zong)(zong)合(he)征(zheng)(慢性(xing)嗜酸性(xing)細(xi)(xi)胞(bao)性(xing)肺(fei)炎(yan)),胸片上(shang)常(chang)出現特征(zheng)性(xing)的肺(fei)周圍部浸潤;和變應(ying)性(xing)肉芽腫病(Churg-Strauss綜(zong)(zong)合(he)征(zheng),一種(zhong)結節(jie)性(xing)多關(guan)節(jie)炎(yan)伴(ban)肺(fei)病變).單純性(xing)嗜酸性(xing)細(xi)(xi)胞(bao)性(xing)肺(fei)炎(yan)(LÖffler綜(zong)(zong)合(he)征(zheng))偶可合(he)并哮喘.
不伴(ban)哮喘的(de)(de)嗜(shi)(shi)酸(suan)(suan)性(xing)(xing)(xing)(xing)(xing)(xing)(xing)細(xi)(xi)(xi)胞(bao)性(xing)(xing)(xing)(xing)(xing)(xing)(xing)肺(fei)(fei)炎(yan)包括急(ji)性(xing)(xing)(xing)(xing)(xing)(xing)(xing)嗜(shi)(shi)酸(suan)(suan)性(xing)(xing)(xing)(xing)(xing)(xing)(xing)細(xi)(xi)(xi)胞(bao)性(xing)(xing)(xing)(xing)(xing)(xing)(xing)肺(fei)(fei)炎(yan),嗜(shi)(shi)酸(suan)(suan)性(xing)(xing)(xing)(xing)(xing)(xing)(xing)細(xi)(xi)(xi)胞(bao)性(xing)(xing)(xing)(xing)(xing)(xing)(xing)肌痛(tong)(tong)綜合(he)征和(he)(he)嗜(shi)(shi)酸(suan)(suan)性(xing)(xing)(xing)(xing)(xing)(xing)(xing)細(xi)(xi)(xi)胞(bao)增(zeng)多(duo)綜合(he)征.急(ji)性(xing)(xing)(xing)(xing)(xing)(xing)(xing)細(xi)(xi)(xi)胞(bao)性(xing)(xing)(xing)(xing)(xing)(xing)(xing)肺(fei)(fei)炎(yan)是一種原因不明的(de)(de)不同病種,可引起急(ji)性(xing)(xing)(xing)(xing)(xing)(xing)(xing)發熱,嚴(yan)重(zhong)低氧血(xue)癥,彌(mi)漫性(xing)(xing)(xing)(xing)(xing)(xing)(xing)肺(fei)(fei)浸(jin)潤(run)(run)以及(ji)支氣管肺(fei)(fei)泡灌洗液中的(de)(de)嗜(shi)(shi)酸(suan)(suan)性(xing)(xing)(xing)(xing)(xing)(xing)(xing)細(xi)(xi)(xi)胞(bao)比例>25%.經皮質激素治療可迅速,徹底緩解.嗜(shi)(shi)酸(suan)(suan)性(xing)(xing)(xing)(xing)(xing)(xing)(xing)細(xi)(xi)(xi)胞(bao)性(xing)(xing)(xing)(xing)(xing)(xing)(xing)肌痛(tong)(tong)綜合(he)征與食(shi)(shi)用(yong)大量的(de)(de)作為(wei)食(shi)(shi)物添加(jia)劑的(de)(de)L-色氨酸(suan)(suan)有(you)關.肺(fei)(fei)浸(jin)潤(run)(run)偶(ou)可伴(ban)有(you)肌痛(tong)(tong),肌無力(li),皮疹和(he)(he)類(lei)似于硬皮病的(de)(de)軟組織硬結.嗜(shi)(shi)酸(suan)(suan)性(xing)(xing)(xing)(xing)(xing)(xing)(xing)細(xi)(xi)(xi)胞(bao)增(zeng)多(duo)綜合(he)征的(de)(de)診斷標準為(wei)持續性(xing)(xing)(xing)(xing)(xing)(xing)(xing)嗜(shi)(shi)酸(suan)(suan)性(xing)(xing)(xing)(xing)(xing)(xing)(xing)細(xi)(xi)(xi)胞(bao)增(zeng)多(duo)(>1500/mm3)達6個月以上,缺乏其他引起嗜(shi)(shi)酸(suan)(suan)性(xing)(xing)(xing)(xing)(xing)(xing)(xing)細(xi)(xi)(xi)胞(bao)增(zeng)多(duo)的(de)(de)病因,累(lei)及(ji)心,肝,脾,中樞(shu)神經系統(tong)或肺(fei)(fei),心臟最常受(shou)累(lei).發熱,體(ti)重(zhong)減輕和(he)(he)貧(pin)血(xue)也常見,常可發生動脈而(er)非(fei)靜脈性(xing)(xing)(xing)(xing)(xing)(xing)(xing)血(xue)栓栓塞疾病。
特征性改變包(bao)括嗜(shi)酸性細(xi)胞(bao)(bao),大(da)單(dan)核(he)(he)細(xi)胞(bao)(bao)充(chong)滿肺泡,肺泡間隔內(nei)(nei)嗜(shi)酸性細(xi)胞(bao)(bao),漿細(xi)胞(bao)(bao)和大(da),小單(dan)核(he)(he)細(xi)胞(bao)(bao)浸潤.也可有細(xi)支氣(qi)管內(nei)(nei)粘(zhan)液填塞和血管浸潤。
癥(zheng)狀和(he)體征(zheng)可輕(qing)微,也可危及生(sheng)命.可伴低(di)度(du)發(fa)熱,輕(qing)度(du)(如有)呼(hu)吸(xi)道癥(zheng)狀,可即時恢(hui)復.其(qi)他類(lei)(lei)型(xing)的PIE綜合征(zheng),可有發(fa)熱和(he)支氣管哮(xiao)喘癥(zheng)狀,包括咳(ke)嗽,喘鳴和(he)靜(jing)息時呼(hu)吸(xi)困(kun)難.如不(bu)治療(liao),慢性(xing)嗜(shi)(shi)酸性(xing)細胞性(xing)肺炎常(chang)進行性(xing)發(fa)展至危及生(sheng)命,類(lei)(lei)似急性(xing)嗜(shi)(shi)酸性(xing)肺炎.常(chang)有明(ming)顯(xian)增高的嗜(shi)(shi)酸性(xing)細胞(20%~40%,有時更高).胸部(bu)X線表現(xian)為不(bu)同肺葉內有迅速(su)出現(xian)及迅速(su)消失的浸(jin)潤病(bing)灶(游走性(xing)浸(jin)潤)。
根據病(bing)(bing)(bing)人(ren)居住(zhu)的地(di)理區域尋找(zhao)致病(bing)(bing)(bing)蠕(ru)蟲,痰內可找(zhao)到(dao)寄生(sheng)蟲和(he)煙(yan)曲霉,需仔細詢問用藥史.鑒別診(zhen)斷包括結核,結節(jie)病(bing)(bing)(bing),霍奇金病(bing)(bing)(bing)和(he)其他淋巴組(zu)織增(zeng)生(sheng)性(xing)(xing)疾(ji)(ji)病(bing)(bing)(bing),肺嗜(shi)酸性(xing)(xing)細胞性(xing)(xing)肉芽(ya)(ya)腫,脫屑性(xing)(xing)間質性(xing)(xing)肺炎和(he)膠原性(xing)(xing)血(xue)管疾(ji)(ji)病(bing)(bing)(bing)。過敏性(xing)(xing)肺炎和(he)Wegener肉芽(ya)(ya)腫常不伴嗜(shi)酸性(xing)(xing)細胞增(zeng)多。
本病可為自限性(xing),呈良性(xing),可不需治(zhi)(zhi)療.如癥狀(zhuang)嚴重,使(shi)用(yong)皮質類(lei)固醇(chun)常有極(ji)好效果;對(dui)于急性(xing)嗜酸性(xing)細胞肺炎(yan)和特發(fa)性(xing)慢性(xing)嗜酸性(xing)肺炎(yan)者該(gai)治(zhi)(zhi)療可挽救(jiu)生命。如出(chu)現支(zhi)氣管哮(xiao)喘(chuan),采用(yong)通常治(zhi)(zhi)療方(fang)法.有蠕(ru)蟲感染的,應使(shi)用(yong)驅蟲藥(yao)。