DiffuseCysticLungDisease

Diffuse Cystic Lung Disease,杜倩妮2015-12-3,概述,临床表现：无特异性，常表现为慢性咳嗽、气促等，部分患者以复发性自发性气胸为特异的临床表现，少数患者因常规胸部CT或HRCT检查而发现。影像学表现：肺实质内弥漫性分布多个囊性病变，囊状影通常壁厚薄不一（常1cm,壁厚一般1mm,偶尔也可达2-3mm。病理证实囊壁由上皮细胞和/或纤维组织构成。,概念-肺类囊性病变,CT上表现为肺部异常含气透亮影改变，如肺空洞，小叶中央型肺气肿、囊性支气管扩张以及蜂窝肺等。,Cavities,Cavities are intraparenchymal air-filled lucencies with definable walls that are thicker (4 mm) than those of lung cysts. Common causes of cavities are broncho-genic carcinoma, lung metastasis, vasculitis, and infectious diseases such as lung abscess, septic emboli,tuberculosis, and fungal infection.,Emphysema,Emphysema is abnormal, permanent enlargement of airspaces distal to the terminal bronchiole, accompanied by the destruction of their walls.,Bronchiectasis,Bronchiectasis is defined as focal or diffuse, irreversible dilatation of the bronchial tree. The most common cause is recurrent pulmonary infection, but it can also be part of hereditary . Bronchiectasis can be differentiated by following the dilated airway branching from the pulmonary cyst on multiple sequential chest CT scans. This branching pattern is better recognized on coronal or sagittal reconstructed images.Bronchiectasis is usually associated with bronchial wall thickening, centrilobular nodules, and air-trapping.,Honeycombing,Honeycombing is multiple rows of air-filled lucencies with variable wall thickness that are clustered in the subpleural area. Honeycombing is associated with end-stage pulmonary fibrosis, predominantly distributed in the lower lobes and usually 1 cm in diameter.Other features of pulmonary fibrosis, such as decreased lung volume, reticular opacities, architectural distortion,and traction bronchiectasis, accompany honeycombing.Honeycombing is differentiated from cystic lung disease because multiple air-filled spaces are distributed along the subpleural area, not within the lung parenchyma.,肺囊性病变与类囊性病变的影像学鉴别要点,引起弥漫性肺囊性病变的疾病及分类,淋巴管肌瘤病 散发性； 结节性硬化症相关性郎格汉斯细胞组织细胞增生症卵巢滤泡激素基因相关综合征 BinHoggDub6综合征 家族性自发性气胸淋巴相关性疾病 淋巴细胞性间质性肺炎(原发或继发于结缔组织病) 肺淋巴瘤 淀粉样病变 非淀粉样物质沉积症感染性疾病 肺孢子菌肺炎 复发性呼吸道乳头状瘤 高免疫球蛋白E综合征间质性肺疾病 脱屑性间质性肺炎 过敏性肺炎,最常见的： Pulmonary Langerhans cell histiocytosis (LCH) Lymphangiomyomatosis (LAM),1.朗格汉斯组织细胞增生症（LCH）,病因不明，机制不清与吸烟显著相关好发年龄20-40岁，男性发病率稍高于女性可累及全身多个器官，也可仅累及肺（PLCH）预后良好，可自行或激素治疗后或戒烟后缓解,1.朗格汉斯组织细胞增生症（LCH）,双肺气囊腔样病变和小结节影，以中上肺分布为主,很少累及下叶及肋膈角；气囊腔厚薄不规则，一般较薄，直径大小不一，通常小于10mm,形态奇异, 可见 哑 铃 形、分 支 形 气 囊 腔等；结节直径1-5mm，以小叶中央型结节影为主，结节边缘不规则， 结节中心可由空洞形成,空洞性结节可逐渐演变成囊状病变;,42y，M，咳嗽2月余，胸闷、气喘3天，吸烟，每天1包*20年同一患者，图2为戒烟后2月,图1,图2,2.肺淋巴管肌瘤病（LAM）,好发于育龄期女性病理特征为淋巴管、小血管、小气道及其周围类平滑肌细胞的进行性增生，引起局部管腔结构的狭窄或阻塞病理变化：淋巴管或胸导管阻塞后可引起淋巴管回流障碍，当阻塞的淋巴管破裂可出现乳糜胸、腹腔积液。肺小血管平滑肌增生可导致阻塞远端血管腔淤血扩张甚至破裂出血，导致患者咯血。呼吸性细支气管平滑肌细胞增生可导致小气道局限性狭窄，阻塞的远端的远端肺泡扩大融合，形成肺气肿样改变，胸膜下过度膨胀的肺囊泡破裂可出现自发性气胸。预后不佳,2.