嗜(shi)(shi)酸(suan)性(xing)(xing)細(xi)(xi)胞(bao)性(xing)(xing)肺炎(yan)是(shi)一組病因明(ming)確或(huo)尚未明(ming)確,以嗜(shi)(shi)酸(suan)性(xing)(xing)細(xi)(xi)胞(bao)浸(jin)潤(run)為特點(dian),常(chang)伴(ban)周圍(wei)血嗜(shi)(shi)酸(suan)細(xi)(xi)胞(bao)增多的疾病。有時稱為嗜(shi)(shi)酸(suan)性(xing)(xing)細(xi)(xi)胞(bao)增多性(xing)(xing)肺浸(jin)潤(run)(PIE)綜合征。
嗜(shi)酸性(xing)細(xi)胞性(xing)肺(fei)炎是一組病因(yin)明(ming)確或尚(shang)未明(ming)確,以嗜(shi)酸性(xing)細(xi)胞浸潤為特點,常(chang)伴周圍血嗜(shi)酸細(xi)胞增(zeng)多的(de)疾病.
有(you)時稱為嗜酸性細胞增(zeng)多性肺浸潤(PIE)綜合征。
病因包括寄生(sheng)蟲(chong)(如蛔蟲(chong),弓蛔蚴蟲(chong)和絲蟲(chong)),藥物(如青霉(mei)素(su),對氨基(ji)水楊酸,肼苯噠嗪,呋喃妥因,氯丙嗪,磺胺制劑);化學(xue)過敏(min)(min)物質(zhi)(如蒸氣(qi)形式吸(xi)入的碳化鎳);和真菌(jun)(如煙曲(qu)(qu)霉(mei),它引起變應性支(zhi)氣(qi)管肺曲(qu)(qu)霉(mei)病見(jian)后述(shu)).盡管疑有(you)過敏(min)(min)性機制,但大多(duo)數嗜酸性細(xi)(xi)胞(bao)肺炎病因不明.嗜酸性細(xi)(xi)胞(bao)增(zeng)多(duo)提(ti)示為(wei)第(di)Ⅰ型過敏(min)(min)反應,綜合征的其他特點(dian)(血管炎,圓細(xi)(xi)胞(bao)浸潤)提(ti)示為(wei)第(di)Ⅲ型亦可能為(wei)第(di)Ⅳ型反應.
嗜(shi)酸(suan)性(xing)(xing)(xing)細胞(bao)(bao)(bao)(bao)性(xing)(xing)(xing)肺(fei)炎(表76-3)常合(he)并(bing)有支氣(qi)管哮喘.合(he)并(bing)哮喘及(ji)病因不明的嗜(shi)酸(suan)性(xing)(xing)(xing)細胞(bao)(bao)(bao)(bao)性(xing)(xing)(xing)肺(fei)炎可(ke)分(fen)為三類:外源性(xing)(xing)(xing)支氣(qi)管哮喘伴PIE綜(zong)合(he)征(zheng),事(shi)實(shi)上(shang),常為變應性(xing)(xing)(xing)支氣(qi)管肺(fei)曲(qu)霉病;內源性(xing)(xing)(xing)支氣(qi)管哮喘伴PIE綜(zong)合(he)征(zheng)(慢性(xing)(xing)(xing)嗜(shi)酸(suan)性(xing)(xing)(xing)細胞(bao)(bao)(bao)(bao)性(xing)(xing)(xing)肺(fei)炎),胸片上(shang)常出現特征(zheng)性(xing)(xing)(xing)的肺(fei)周圍部浸潤;和變應性(xing)(xing)(xing)肉芽腫病(Churg-Strauss綜(zong)合(he)征(zheng),一(yi)種結節(jie)性(xing)(xing)(xing)多關節(jie)炎伴肺(fei)病變).單(dan)純(chun)性(xing)(xing)(xing)嗜(shi)酸(suan)性(xing)(xing)(xing)細胞(bao)(bao)(bao)(bao)性(xing)(xing)(xing)肺(fei)炎(LÖffler綜(zong)合(he)征(zheng))偶(ou)可(ke)合(he)并(bing)哮喘.
不伴哮喘的嗜(shi)酸(suan)(suan)(suan)性(xing)(xing)(xing)(xing)(xing)細胞性(xing)(xing)(xing)(xing)(xing)肺(fei)(fei)炎包(bao)括急性(xing)(xing)(xing)(xing)(xing)嗜(shi)酸(suan)(suan)(suan)性(xing)(xing)(xing)(xing)(xing)細胞性(xing)(xing)(xing)(xing)(xing)肺(fei)(fei)炎,嗜(shi)酸(suan)(suan)(suan)性(xing)(xing)(xing)(xing)(xing)細胞性(xing)(xing)(xing)(xing)(xing)肌痛(tong)(tong)綜(zong)(zong)合征(zheng)和(he)嗜(shi)酸(suan)(suan)(suan)性(xing)(xing)(xing)(xing)(xing)細胞增多綜(zong)(zong)合征(zheng).急性(xing)(xing)(xing)(xing)(xing)細胞性(xing)(xing)(xing)(xing)(xing)肺(fei)(fei)炎是一種原因不明的不同病(bing)(bing)種,可(ke)引起急性(xing)(xing)(xing)(xing)(xing)發熱,嚴重低氧(yang)血癥,彌漫(man)性(xing)(xing)(xing)(xing)(xing)肺(fei)(fei)浸潤以及(ji)支氣管肺(fei)(fei)泡(pao)灌洗液中的嗜(shi)酸(suan)(suan)(suan)性(xing)(xing)(xing)(xing)(xing)細胞比例>25%.經皮(pi)質(zhi)激素治療可(ke)迅速,徹底(di)緩解.嗜(shi)酸(suan)(suan)(suan)性(xing)(xing)(xing)(xing)(xing)細胞性(xing)(xing)(xing)(xing)(xing)肌痛(tong)(tong)綜(zong)(zong)合征(zheng)與(yu)食用大量的作(zuo)為(wei)食物添加劑的L-色氨酸(suan)(suan)(suan)有關.肺(fei)(fei)浸潤偶可(ke)伴有肌痛(tong)(tong),肌無力,皮(pi)疹和(he)類(lei)似于(yu)硬皮(pi)病(bing)(bing)的軟組織硬結(jie).嗜(shi)酸(suan)(suan)(suan)性(xing)(xing)(xing)(xing)(xing)細胞增多綜(zong)(zong)合征(zheng)的診(zhen)斷標準為(wei)持續性(xing)(xing)(xing)(xing)(xing)嗜(shi)酸(suan)(suan)(suan)性(xing)(xing)(xing)(xing)(xing)細胞增多(>1500/mm3)達6個月以上,缺乏其(qi)他引起嗜(shi)酸(suan)(suan)(suan)性(xing)(xing)(xing)(xing)(xing)細胞增多的病(bing)(bing)因,累及(ji)心(xin),肝,脾,中樞神(shen)經系統或(huo)肺(fei)(fei),心(xin)臟(zang)最常(chang)受累.發熱,體重減輕和(he)貧血也常(chang)見,常(chang)可(ke)發生(sheng)動脈(mo)而非靜(jing)脈(mo)性(xing)(xing)(xing)(xing)(xing)血栓栓塞(sai)疾病(bing)(bing)。
特征性(xing)(xing)改變包括嗜(shi)(shi)酸性(xing)(xing)細(xi)(xi)(xi)胞(bao),大單核細(xi)(xi)(xi)胞(bao)充滿肺泡,肺泡間隔內嗜(shi)(shi)酸性(xing)(xing)細(xi)(xi)(xi)胞(bao),漿細(xi)(xi)(xi)胞(bao)和(he)(he)大,小(xiao)單核細(xi)(xi)(xi)胞(bao)浸潤.也可有細(xi)(xi)(xi)支(zhi)氣管內粘液填塞(sai)和(he)(he)血管浸潤。
癥狀和(he)(he)體征(zheng)可輕(qing)(qing)微,也可危(wei)及生(sheng)命(ming).可伴低(di)度(du)發熱,輕(qing)(qing)度(du)(如有)呼吸道癥狀,可即時恢(hui)復.其他類型的(de)(de)PIE綜合征(zheng),可有發熱和(he)(he)支氣管哮喘(chuan)癥狀,包括咳嗽,喘(chuan)鳴和(he)(he)靜息(xi)時呼吸困難.如不(bu)治療,慢性(xing)(xing)(xing)嗜酸性(xing)(xing)(xing)細胞性(xing)(xing)(xing)肺炎常進行(xing)性(xing)(xing)(xing)發展至危(wei)及生(sheng)命(ming),類似(si)急性(xing)(xing)(xing)嗜酸性(xing)(xing)(xing)肺炎.常有明顯增高的(de)(de)嗜酸性(xing)(xing)(xing)細胞(20%~40%,有時更(geng)高).胸部X線表現為(wei)不(bu)同(tong)肺葉內有迅速(su)出現及迅速(su)消失的(de)(de)浸潤病灶(zao)(游(you)走性(xing)(xing)(xing)浸潤)。
根據病(bing)(bing)(bing)人居住(zhu)的地理區域尋(xun)找致(zhi)病(bing)(bing)(bing)蠕蟲,痰內可(ke)找到寄生(sheng)蟲和煙曲霉,需(xu)仔(zi)細詢(xun)問用藥史(shi).鑒(jian)別診斷(duan)包括結核,結節病(bing)(bing)(bing),霍奇(qi)金病(bing)(bing)(bing)和其他(ta)淋巴(ba)組織增(zeng)生(sheng)性疾病(bing)(bing)(bing),肺(fei)嗜酸(suan)性細胞(bao)性肉(rou)芽腫(zhong)(zhong),脫(tuo)屑性間(jian)質性肺(fei)炎和膠原性血管疾病(bing)(bing)(bing)。過敏性肺(fei)炎和Wegener肉(rou)芽腫(zhong)(zhong)常不伴嗜酸(suan)性細胞(bao)增(zeng)多。
本(ben)病可(ke)為(wei)自限性,呈良(liang)性,可(ke)不需治療(liao).如癥狀(zhuang)嚴重,使用(yong)皮質(zhi)類固醇常有極(ji)好效果;對于急性嗜酸性細胞肺(fei)炎和特發性慢性嗜酸性肺(fei)炎者該治療(liao)可(ke)挽救生命。如出現支氣(qi)管哮喘,采用(yong)通常治療(liao)方法.有蠕蟲(chong)感染的,應使用(yong)驅蟲(chong)藥。