弥漫性细支气管炎.ppt

Diffuse panbronchiolitis,胡建敏,In 1969, the disease was named DPB to distinguish it from chronic bronchitis “Diffuse“ refers to the distribution of the lesions throughout both lungs “pan-“ refers to the involvement of inflammation in all layers of the respiratory bronchioles,EPIDEMIOLOGY,East Asians, predominantly in Japan usually occurring in the 20-40 yrs 11 cases per 100,000 people no remarkable sex predominance could be observed Two-thirds of patients are nonsmokers and patients have no particular history of inhalation of toxic fumes,associated with sinusitis in 75% of patients etiology is not clear Asian immigrants also suffers. research has shown an association with HLABw54, found predominantly among East Asians,At autopsy, lungs in DPB appear hyperinflated and often show bronchiectasis Cut sections show yellow nodules, 23 mm in diameter, centring on small airways,PATHOLOGY,transmural and peribronchial infiltration at the level of the respiratory bronchioles by lymphoctyes,plasma cells and histocytes accumulation of interstitial foam cells lymphoid hyperplasia most of the alveoli are unaffected ectasia of proximal membranous bronchioles,intraluminal inflammatory exsudates ultimately, widespread bronchiectasis occur,临床表现,咳嗽，咳痰，活动后气促 少数患者可无自觉症状 疾病早期起病隐袭，咳痰无色或白痰 并发感染时痰呈黄色或绿色 后期呼吸困难，活动时明显,影像学表现,胸片的典型表现是两肺弥漫性边缘不清的颗粒状结节影，直径约2-5mm，以两下肺野为著，常伴有肺过度膨胀。70%的病人在初次就诊时胸片即有双肺结节影 。 HRCT 表现： 小叶中心结节,且无相互融合的趋势 树芽征 近侧细支气管继发性扩张，伴感染时管腔内可见粘液栓塞 呼气相CT：外周的空气潴留现象 影像学表现具有特异性，可以提示DPS诊断，但尚不足以确诊，确诊仍需组织形态学的特异性改变。,Chest radiograph：bilateral, diffuse, small nodular shadows with pulmonary hyperinflation,影像学鉴别诊断,DPB起病隐匿, 一般患者均有咳嗽、咳痰、气促多年的呼吸系统病史, 其临床表现缺乏特异性, 早期极易误诊。 支气管扩张(BE) 临床也有咳嗽、咳痰, 长期反复发作的呼吸系统病史。但胸部CT影像学表现为某一叶/段多级支气管的囊环状薄壁透光影和“双轨征”, 多累及较大支气管, 管壁一般不增厚, 严重者常可见支气管黏液嵌塞和/或液平。病变累及范围远不如DPB广, 常可见囊状支气管扩张,虽然也可见“树芽征”, 但多无细粟粒样小结节影伴随。结合临床其他检查资料, 及有无副鼻窦炎病史及副鼻窦CT检查, 当可作出正确诊断。,肺结核支气管播散可有“树芽征”出现, 也是这一CT征象最早被描述的疾病, 但肺结核病灶有多态性的背景特点, 其肺内病变除“树芽征”外, 还伴有斑片影、空洞等多种形态。而急性粟粒性肺结核则表现为细粟粒样结节影, 其密度更淡, 分布更密集, 有大小、密度、分布三均匀的特点, 但无“树芽征”。因此在与DPB的鉴别诊断时胸部CT检查十分必要, 尤其使用高分辨率CT(HRCT)更有利于病变的显示和鉴别。 尘肺的早期影像学表现是双肺弥漫性分布的粟粒样小结节影, 但不呈小叶中心性分布。只有当气道损伤延伸至小气道时, CT上可出现细支气管扩张及小叶中心结节影(即TIB) 。尘肺早期一般不伴发支气管扩张,亦无树芽征和支气管壁增厚。但纵隔、肺门淋巴结增大伴钙化是其特征, 且有生产性粉尘接触史。而DPB患者无粉尘相关职业史, 常无纵隔、肺门淋巴结肿大、钙化。,1. Persistent cough, sputum and exertional dyspnoea 2. History of chronic paranasal sinusitis 3. Bilateral diffuse small nodular shadows on plain chest film or centrilobular micronodules on chest CT 4. Coarse crackles 5. FEV1/FVC 70% and PaO2 80 mmHg 6. Titre of cold haemagglutinin 64,Diagnostic criteria,PROGNOSIS,The prognosis of patients with DPB was poor, with 5- and 10-yr survival rates in 1983 of 62.1 and 33.2%, respectively. Long-term treatment with erythromycin has increased the 10-yr survival rate to 90%.,TREATMENT,low-dose, long-term erythromycin therapy Once treatment is stopped, the patient must