结节病CT典型和不典型型表现.ppt

Pulmonary Sarcoidosis: Typical and Atypical Manifestations at High- Resolution CT with Pathologic Correlation,Recent Advances in Sarcoidosis Chest 2011;139;,结节病CT典型和不典型表现 结节病进展,Organ Lung Pleura Lymph Nodes Skin Eye Nasal Mucosa（鼻粘膜） Larynx（喉） Bone Marrow Spleen Liver,% 90 1-5 75-90 25 25 20 5 15-40 50-60 60-90,Organ Kidney Calcium Metabolism Nervous System Bone Joints Heart Endocrine Glands Parotid Gland Gastrointestinal System,% Rare 1-2 5 5 25-50 5 Rare 10 Rare,Sarcoidosis is a multisystem disorder that is characterized by noncaseous epithelioid cell granulomas, which may affect almost any organ. 结节病是一种以非干酪样坏死的上皮细胞肉芽肿为特点的多系统疾病，几乎可以累及所有器官。

Sarcoidosis may be asymptomatic or chronic. It commonly improves or clears up spontaneously（自愈或好转）. More than 2/3 of people with lung sarcoidosis have no symptoms after 9 years.About 50% have relapses. About 10% develop serious disability.,Sarcoidosis of the lung is primarily an interstitial lung disease in which the inflammatory process involves the alveoli, small bronchi, and small blood vessels.,Typical and Atypical Features of Pulmonary Sarcoidosis at High-Resolution CT Typical features 1 Lymphadenopathy: hilar, mediastinal (right paratracheal), bilateral, symmetric, and well defined 2 Nodules: micronodules (2–4 mm in diameter; well defined, bilateral); macronodules (≥5 mm in diameter, coalescing) 3 Lymphangitic spread: peribronchovascular, subpleural, interlobular septal 4 Fibrotic changes: reticular opacities, architectural distortion, traction bronchiectasis, bronchiolectasis, volume loss Bilateral perihilar opacities 5 Predominant upper- and middle-zone locations of parenchymal abnormalities,结节病原本是肺间质性病变，累及肺泡，支气管和小血管,淋巴结增大，两侧对称，境界清楚,大小结节,淋巴管播散，支气管血管鞘，胸膜下，小叶间隔,纤维化改变，网状阴影，肺结构扭曲，牵拉性支扩，肺容积缩小，两肺门旁致密影,中上肺为主,典型特征,Atypical features 1 Lymphadenopathy: unilateral, isolated, anterior and posterior mediastinal 2 Airspace consolidation: masslike opacities, conglomerate masses, solitary pulmonary nodules, confluent alveolar opacities (alveolar sarcoid pattern) 3 Ground-glass opacities4 Linear opacities: interlobular septal thickening, intralobular linear opacities,5 Fibrocystic changes: cysts, bullae, blebs, emphysema, honeycomb-like opacities with upper- and middle-zone predominance,单侧孤立前后纵隔淋巴结增大,肺泡实变：肿块，实质性结节，实变融合,毛玻璃阴影,线状阴影小叶间隔增厚小叶年线状阴影,不典型表现,囊性纤维化改变：囊，大泡，小泡，肺气肿，蜂窝样改变,6 Miliary opacities粟粒样改变7 Airway involvement: mosaic attenuation pattern, tracheobronchial abnormalities, atelectasis 气道累及：马赛克改变，气管支气管异常，肺不张8 Pleural disease: effusion, chylothorax, hemothorax, pneumothorax, pleural thickening, calcification Pleural plaquelike opacities9 Mycetoma, aspergilloma 霉菌球,During the past decade, advances have been made in the study of sarcoidosisThe Mycobacterium tuberculosis catalase-peroxidase (mKatG) protein, a potential antigen,has been identified, 结核分枝杆菌过氧化氢酶过氧化物酶（mkatg）蛋白，作为一个潜在的抗原已被确定。

PET scanning has proven valuable in locating occult sites for diagnostic biopsy. PET扫描已被证明在定位活检诊断隐匿性病灶的价值Endobronchial ultrasound- guided transbronchial needle aspiration (EBUS-TBNA) of mediastinal lymph nodes has facilitated diagnosis, often eliminating the need for more-invasive procedures, such as mediastinoscopy. 支气管内超声引导下经支气管针吸活检（EBUS-TBNA）的 纵隔淋巴结有利于诊断，往往避免更具侵袭性的操作，如纵隔镜Gene expression analyses have more clearly defined cytokine dysregulation in sarcoidosisCurrently, no reliable prognostic biomarkers have been identified. 目前，还没有可靠的预后标志物已被确定。

The tumor necrosis factor (TNF) inhibitors, a relatively new class of steroid-sparing agents, have been used in patients with refractory disease. It is unclear whether medications used to treat pulmonary arterial hypertension are safe and effective for the treatment of sarcoidosis-associated pulmonary hypertension. 肿瘤坏死因子（TNF）抑制剂，一个相对较新的类固醇保代理类药品，已被用于治疗难治性疾病目前还不清楚是否用于治疗肺动脉高压的药物治疗结节病相关性肺动脉高压是安全和有效的Pathologic Correlation 相关病理Granulomas in the lung parenchyma have a characteristic distribution in relation to lymphatics in the peribronchovascular interstitial space, subpleural interstitial space, and, to a lesser extent, the interlobular septa (ie, a lymphangitic distribution)肺实质肉芽肿分布与支气管血管鞘，胸膜下结缔组织，小叶间隔中淋巴管相关. Thickened bronchovascular bundles and small perivascular nodules seen at CT corresponded to granulomas within the connective tissue sheath surrounding pulmonary airways and vessels. Pleural or subpleural nodules were correlated with granulomas adjacent to the visceral pleuraCT上支气管血管鞘增厚和小结节是与包绕气道血管结缔组织鞘中肉芽肿，胸膜和胸膜下结节与 脏层胸膜旁肉芽肿相关。

Ground-glass opacities represented an accumulation of many granulomatous lesions, with or without fibrosis, in the alveolar septa and around the small vessels. No alveolitis was seen肺泡间隔小血管周围大量肉芽肿是毛玻璃阴影主要原因，可伴纤维化，但没有肺泡炎Large parenchymal nodules (>1 cm in diameter) represented coalescent granulomas大结节是肉芽肿病变的融合Air bronchiolograms within regions of dense consolidation on CT images corresponded to bronchiolar dilatation with surrounding fibrosis 支气管充气症是纤维化旁的支扩honeycomb-like pattern of microscopic cysts seen at pathologic analysis. 蜂窝样改变在显微镜下就是很多的小囊,。