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 imagesAdditional 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:
Differential diagnosis 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 pointsProgressive 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:
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:
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: 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。
|