慢性嗜酸细胞性肺炎4例报道并文献复习

发布时间：2018-05-15 23:28

本文选题：嗜酸性粒细胞 + 原因不明　；参考：《广西医科大学》2017年硕士论文

【摘要】：目的:探讨慢性嗜酸细胞性肺炎(CEP)的临床诊疗过程及其特征,提高对CEP的认识。方法:回顾性分析2008年1月至2016年9月广西医科大学第一附属医院收治的经实验室检验、结合影像资料及病理结果最终诊断原因尚不清楚的4例慢性嗜酸性肺炎患者的临床资料,并检索中知、万方、Pubmed等数据库,对相关文献进行系统性分析,归纳其临床特点。结果:(1)我院病例资料:(1)4例患者,男:女1:1,其中有吸烟史2例,年龄26~67岁,病程3月~4年。起病亚急性或慢性,无肺外其他系统症状,4例均有咳嗽,咳少量白痰、活动后气促2例,喘息、胸痛、胸闷各1例,发热、乏力、体重下降各1例。1例闻及湿Up音。(2)4例患者均行自身抗体谱、抗链球菌溶血素O、血沉、补体、常规生化、肌酶谱、寄生虫、病原学、肿瘤抗原、T细胞亚群等检查,并结合病史,排除了继发性EOS增多、肺以外器官系统EOS浸润和伴有EOS增多的其他间质性肺病。4例患者曾全部误诊,分别为“支气管哮喘、支气管炎、细菌性肺炎、肺结核”,起病1月~2年后才确诊。(3)3例患者外周血EOS增高(1.01~3.34×10~9/L),2例外周血白细胞总数升高、轻度贫血、低氧血症,3例ESR增快、4例CRP升高,1例骨髓细胞学见成熟嗜酸细胞增多、未见原始细胞,F/P融合基因阴性。(4)4例纤支镜镜下均可见支气管粘膜充血,2例管腔内见脓性分泌物。2例行肺泡灌洗液细胞分类计数检查,分别为EOS比例增高(30%)、EOS计数升高(0.0217×10~9/L)。4例tblb均见较多eos浸润,亦见到伴淋巴细胞浸润,其中2例肺间质肿胀、肺泡上皮轻度增生。(5)肺ct示磨玻璃影4例,斑片状、条索状实变影3例,不均匀斑片状致密影、空洞、病灶游走各2例。4例均为双肺受累,3例以上肺为主,3例病变主要位于肺周边、胸膜下。(6)3例患者做肺功能检查,弥散障碍2例,混合性以阻塞为主通气障碍1例,通气功能正常2例。(7)4例均口服糖皮质激素治疗,疗程2月~4年,1周内症状改善,1~2周外周血eos恢复正常,1周~2月复查肺ct病灶明显吸收,后续随访中3例完全吸收,另1例激素减量过程中复发2次、病灶游走、但总体上明显吸收。仅1例复查肺功能,提示弥散功能好转。(2)中英文文献复习结果:1975年至今仅70例报道资料较完整,肺部有阴影、balf或肺活检均证实eos浸润,且排除继发性、肿瘤、自身免疫性疾病、肺外器官eos浸润的疾病及其他间质性肺病后诊断慢性嗜酸性肺炎,多为个案报道。(1)年龄跨度大(1~79岁),平均年龄50.13岁,40~69岁为高峰,仅3例有吸烟史,男:女26:35。19例确诊前有误诊,14例既往有哮喘病史。仅1例急性起病,余亚急性或慢性,无肺外其他系统症状,以喘息、气促、咳痰,伴发热、盗汗、体重下降为主要表现;仅3例有急性呼吸衰竭、需机械通气,22例肺部有Up音、1例有杵状指。(2)87.7%外周血eos比例升高,8例骨髓均见成熟eos增多,未见幼稚细胞,分别有1例提供f/p、jak2基因结果提示阴性,1例染色体核型正常。54例做纤支镜检查,8例镜下见粘液痰栓、2例见支气管壁白色小结节,balf中eos比例全部升高(平均49.1%)。50例肺活检均可见到肺泡、肺间质eos浸润,6例见轻度纤维化、机化。34例提供肺功能结果,弥散障碍68.4%,通气功能可呈阻塞性、限制性、混合性或正常,其中阻塞性47.1%、较多见于既往有哮喘病史患者。(3)60%以上患者影像学表现为双上肺、外周分布为主、多发实变、磨玻璃影,13.4%见病灶游走。(4)确诊后除4例以单一ics作为初始治疗外,66例患者口服相当于强的松0.3~2.0mg/kg.d开始治疗,其中62例为0.5~1.0mg/Kg.d,疗程平均9月,最长11年。4例单一ICS治疗患者病情进展后改用口服激素治疗症状及影像学均改善。(5)预后良好,仅1例因冠心病突发死亡,激素减量或停药复发有12次,8次见于开始治疗1月~6月停药患者。复发后重新激素治疗均再次好转。结论:(1)嗜酸细胞浸润肺组织,经过常规生化、病原学、免疫抗原抗体等检查,排除继发因素、无肺外器官累及,原因尚不明确,需考虑慢性嗜酸细胞性肺炎可能。(2)骨髓融合基因检测有助于排除克隆性骨髓增殖性疾病,其在慢性嗜酸细胞性肺炎的诊断程序中需要更多的研究进一步探讨。(3)慢性嗜酸细胞性肺炎病程长、预后良好,40~69岁为发病高峰年龄,以咳嗽、气促喘息为主要表现,可伴发热、盗汗、体重下降等全身症状,无胸外症状、易误诊。(4)影像学超过60%患者表现为双上肺、外周分布为主的多发斑片状实变、磨玻璃影,16.6%见病灶游走。(5)首选口服糖皮质激素治疗,建议强的松0.5~1mg/kg.d为初始剂量,疗程9月左右,不应少于6月,6月内停药或激素减量易复发,复发后激素仍然敏感,可酌情减慢减量过程、延长疗程。
[Abstract]:Objective: To explore the clinical diagnosis and treatment process of chronic eosinophilic pneumonia (CEP) and its characteristics, and to improve the understanding of CEP. Methods: a retrospective analysis of 4 cases of chronic acidophilia in the First Affiliated Hospital of Guangxi Medical University from January 2008 to September 2016, combined with the image data and the final diagnosis of pathological results, was not clear. The clinical data of the pneumonia patient, and retrieved knowledge, Wanfang, Pubmed and other databases, systematically analyzed the related literature, and summed up its clinical characteristics. Results: (1) the case data of our hospital: (1) 4 cases, male: female 1:1, among them, there were 2 cases of smoking history, age 26~67 years, the course of disease in March ~4. The onset of subacute or chronic disease, no other systemic symptoms of the lung, 4 cases were all There were 2 cases of cough, cough, white sputum, 2 cases of breath, chest pain, chest tightness in 1 cases, fever, fatigue, and weight loss in 1 cases of.1 cases and wet Up sound. (2) all 4 patients received autoantibody spectrum, anti Streptococcus hemolysin O, erythrocyte sedimentation, complement, routine biochemistry, muscle enzyme spectrum, parasite, etiological, tumor antigen, T cell subgroup, etc. In addition to secondary EOS increase, EOS infiltration of the organ system outside the lung and other interstitial lung diseases with EOS increase in.4 were all misdiagnosed as "bronchial asthma, bronchitis, bacterial pneumonia, pulmonary tuberculosis", and the disease was confirmed only after ~2 years in January. (3) 3 patients had increased peripheral blood EOS (1.01~3.34 x 10~9/L), and 2 cases of peripheral leukocyte total. Increase in number, mild anemia, hypoxemia, 3 cases of ESR faster, 4 cases of CRP increase, 1 cases of bone marrow cytology to see more eosinophil, no original cells, F/P fusion gene negative. (4) 4 cases of bronchoscopy under the bronchoscopy can show the congestion of bronchial mucosa, 2 cases of purulent secretory substance in the tube.2 routine alveolar lavage cell classification count, respectively, EOS ratio, respectively. Cases increased (30%), EOS count increased (0.0217 x 10~9/L).4 cases, TBLB showed more EOS infiltration and also accompanied by lymphocytic infiltration, including 2 cases of pulmonary interstitial swelling and mild hyperplasia of alveolar epithelium. (5) 4 cases of lung CT grind glass shadow, 3 cases of patchy, streaklike solid shadow, nonuniform patch shape density shadow, cavity, and 2 cases of.4 cases in each of the lesions, each of the lesions, all.4 cases were double lung involvement, 3. 3 Above lung, 3 cases were mainly located in the periphery of the lung, under the pleura. (6) 3 patients had pulmonary function examination, 2 cases of diffusion barrier, 1 cases with obstructive ventilatory obstruction and 2 cases of normal ventilation. (7) 4 cases were treated with glucocorticoid, the course of treatment was improved in ~4 years in February, the symptoms were improved in 1 weeks, and the peripheral blood EOS was restored to normal in 1~2 weeks, and CT of 1 weeks ~2 month was rechecked CT lung CT. Lung CT was rechecked 1 weeks lung CT lung CT check lung CT review lung CT check lung CT review lung CT for ~2 month reexamination lung CT The lesions were absorbed obviously, 3 cases were completely absorbed in follow-up and 2 times in 1 cases of hormone reduction, and the lesion wandering, but overall obvious absorption. Only 1 cases rechecked pulmonary function, suggesting that diffusion function improved. (2) the literature review in Chinese and English Literature: from 1975 to date, only 70 cases were reported to be more complete, lung had shadow, BALF or lung biopsy confirmed EOS immersion Moistening, and excluding secondary, tumor, autoimmune disease, EOS infiltration of extrapulmonary organs and other interstitial lung disease diagnosis of chronic eosinophilic pneumonia. (1) the age span is large (1~79 years old), the average age is 50.13 years old, 40~69 is the peak, only 3 cases have smoking history, male: 26:35.19 cases have misdiagnosis before diagnosis, 14 cases have wheeze. The history of wheezing. Only 1 cases of acute onset, Yu Ya acute or chronic, no other systemic symptoms of lung, breathing, breath, expectoration, fever, sweating, weight loss, only 3 cases with acute respiratory failure, mechanical ventilation, 22 cases of Up in the lungs, 1 cases of clubbing. (2) 87.7% peripheral blood EOS ratio increased, 8 cases of bone marrow and mature EOS increased, and no bone marrow. There were 1 cases of naive cells, 1 cases were provided with f/p, JAK2 gene results were negative, 1 cases of chromosome karyotype normal.54 cases, 8 cases with mucous phlegm thrombus, 2 cases of white small nodules in bronchial wall, and all EOS ratio in BALF (mean 49.1%),.50 cases of lung biopsy showed pulmonary alveolus, pulmonary interstitial EOS infiltration, 6 case mild fibrosis, machine .34 cases provided the results of pulmonary function, diffusion barrier 68.4%, ventilation function can be obstructive, restrictive, mixed or normal, of which obstructive 47.1%, more frequently seen in patients with past history of asthma. (3) more than 60% of the patients were characterized by double upper lung, peripheral distribution, multiple real changes, glass shadow, 13.4% lesions wandering. (4) 4 cases (4) 4 cases except 4 cases except 4 cases, except 4 cases after diagnosis except after diagnosis except 4 cases except 4 cases except 4 cases except after diagnosis after diagnosis. With a single ICs as the initial treatment, 66 patients were given the equivalent of prednisone 0.3~2.0mg/kg.d, of which 62 cases were 0.5~1.0mg/Kg.d, the average course of treatment was September, the longest 11 years of.4 patients with single ICS treatment were improved by oral hormone treatment and imaging. (5) the prognosis was good, only 1 cases died of CHD sudden death. There were 12 times of reduced or stopped drug recurrence, 8 times in the beginning of the treatment of ~6 months in January. After relapse, re hormone treatment was all better again. Conclusion: (1) eosinophil infiltration of lung tissue, after routine biochemical, pathogenic, immuno antigen antibody examination, excluding secondary factors, no external pulmonary organ involvement, the reason is not clear, need to consider chronic eosinophilia. Cytosolic pneumonia may. (2) bone marrow fusion gene detection helps to exclude cloned myeloproliferative diseases, and it needs further study in the diagnosis of chronic eosinophilic pneumonia. (3) chronic eosinophilic pneumonia has a long course, good prognosis, 40~69 age as the peak age, cough and gaspant breath as the main table. Now, it can be accompanied by fever, night sweating, weight loss and other systemic symptoms, without external symptoms of chest, easy to be misdiagnosed. (4) more than 60% of the patients with imaging findings are double upper lung, the peripheral distribution of multiple patchy changes, glass shadow and 16.6% of the lesions travel. (5) the first choice of oral glucocorticoid therapy, recommended prednisone 0.5~1mg/kg.d as the initial dose, about September, course of treatment, It should not be less than June. In June, drug withdrawal or hormone reduction is easy to relapse. Hormone is still sensitive after relapse.

【学位授予单位】：广西医科大学
【学位级别】：硕士
【学位授予年份】：2017
【分类号】：R563.1

【相似文献】