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双语病例丨进行性大量肺纤维化(复杂矽肺)X/CT

 zskyteacher 2018-06-29


History: A 68-year-old woman presents to the emergency room with acute urinary tract infection symptoms. Chest radiography was performed based on the patient's history of fever. 

病史:68岁女性,急性尿路感染就诊,因其发热病史行胸部平片检查。


Posteroanterior and lateral chest radiographs are shown below. 

胸部正侧位片如下所示。


CT images

Additional history: Upon further questioning, the patient describes years of chronic cough and progressive dyspnea on exertion. She also states that she worked as a stone cutter for 30 years. 

病史补充:进一步询问发现患者慢性咳嗽数年,劳累性进行性呼吸困难,曾做石匠工作约30年。


An unenhanced CT scan of the chest was performed. Axial views of the upper thorax in lung, bone, and soft-tissue (hilar level) windows are shown below.

行胸部CT平扫,上胸部肺窗、骨窗、软组织窗(肺门水平)如下所示。


Findings

  • Chest radiography: Bilateral apical masses are seen with associated bilateral hilar retraction. Subtle hilar calcifications also are noted. 

  • Chest CT: CT demonstrates bilateral apical soft-tissue masses, with the right apical mass containing calcifications. There is surrounding fibrosis and associated subtle surrounding emphysema. Eggshell calcifications are noted within the bilateral hilar lymph nodes. 


影像表现:

  • 胸部平片:可见双肺尖肿物伴双肺门收缩,肺门淋巴结见多发细微钙化。

  • 胸部CT:CT示双肺尖软组织肿块,右肺尖肿物内见钙化,周围可见纤维化和肺气肿。 双侧肺门淋巴结可见蛋壳样钙化


Differential diagnosis

  • Progressive massive fibrosis (complicated silicosis)

  • Pulmonary sarcoidosis

  • Tuberculosis

  • Malignancy

  • Pulmonary talcosis

鉴别诊断:

  • 进行性大量肺纤维化(复杂矽肺)

  • 肺结节病

  • 肺结核

  • 恶性肿瘤

  • 滑石肺


Diagnosis: Progressive massive fIbrosis (complicated silicosis)

最后诊断:进行性大量肺纤维化(复杂矽肺)



Key points

Progressive massive fibrosis (complicated silicosis)


Pathophysiology

  • Progressive massive fibrosis develops from the confluence and expansion of individual silicotic nodules.

  • Histopathological analysis reveals a large conglomerate with multiple foci of central hyalinized cartilage, surrounding pigmented rim of macrophages, central focal necrosis, and occasional granulomatous inflammation.

病理生理学:

  • 进行性的大量肺纤维化是从个别矽肺结节的汇合和扩张发展而来;

  • 组织病理学分析发现其为一个大的团状集合物,由中央有多发透明软骨灶,周围有色素沉着的巨噬细胞环,中央局灶性坏死和偶发的肉芽肿性炎症组成。


Epidemiology

  • A broad range of occupations involve exposure to silica, including mining, stone cutting, masonry, steelwork, construction, sandblasting, glass manufacturing, and cement production.

  • Up to 200,000 miners and 1.7 million nonmining workers in the U.S. have experienced significant silica exposure.

  • One-third of hard rock miners have radiographic evidence of silicosis.

  • Due to improvements in employee protection, the mortality associated with silicosis has significantly decreased in the U.S. over the past 30 years.

流行病学:

  • 与暴露在二氧化硅的相关职业有关,包括采矿,石材切割,砌体,钢结构,建筑,喷砂,玻璃制造和水泥生产;

  • 在美国,有高达20万名矿工和170万非矿工工人经历了显着的二氧化硅暴露;

  • 三分之一的硬岩矿工有矽肺的影像学证据;

  • 由于员工保护水平的提高,过去30年来美国矽肺病死亡率显著下降。


Clinical presentation

  • Variable clinical presentation is seen.

  • The condition slowly develops, often manifesting 10 to 30 years after first exposure.

  • Common symptoms include chronic cough, dyspnea on exertion, and sputum production.

  • Symptoms are often more severe in progressive massive fibrosis and worsen with the progression of radiographic abnormalities.

临床表现:

  • 临床表现各不相同;

  • 病情发展缓慢,通常在首次暴露后10至30年表现出来

  • 常见症状包括慢性咳嗽,运动时呼吸困难和咳痰

  • 进行性大量纤维化的症状通常更为严重,并随着影像异常的进展而恶化。



Imaging features

Chest radiograph and CT general findings:

  • Bilateral symmetrical soft-tissue masses/nodules are seen (> 1 cm) with irregular margins. Large opacities are the result of nodule coalescence.

  • Predominantly appear in the upper lung zone.

  • Lateral margins are often parallel with the chest wall.

  • May contain amorphous calcifications.

  • May cavitate due to central necrosis.

  • Gradual migration of masses toward the hilum, resulting in surrounding areas of emphysema.

  • Eggshell calcifications may be seen in hilar and mediastinal adenopathy.

FDG PET/CT: May be FDG-avid and mimic malignancy.


影像表现

胸片及CT的主要表现:

  • 双侧对称性软组织肿物或结节,直径大于1cm,边缘不规则,结节融合后可表现为大片状阴影;

  • 主要见于肺上叶;

  • 外侧缘常平行于胸壁;

  • 可包含不定形的钙化;

  • 由于中心坏死可出现空洞;

  • 病变逐渐移向肺门,导致周围区域肺气肿;

  • 肺门及纵隔淋巴结可见蛋壳样钙化。

FDG PET/CT:可见FDG摄取,类似恶性表现。


Treatment

  • There is no proven specific therapy.

  • Avoidance of further silica exposure is necessary.

  • Supportive therapy as needed is recommended.

  • Lung transplantation has been successfully utilized.

治疗:

  • 无特别有效的具体治疗方法;

  • 避免进一步的二氧化硅暴露非常必要;

  • 根据需要进行支持治疗;

  • 肺移植已得到成功应用。


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