每日一题_2024.02.22

A 28-year-old woman presented to the hospital with a 6-month history of dry cough. She was a lifetime nonsmoker and reported no fevers, joint aches, eye pain, or rashes. On physical examination, auscultation of both lower lungs revealed fine crackles. High-resolution computed tomography (CT) of the chest showed mediastinal lymphadenopathy and diffuse ground-glass opacities (left, axial view). Also visible were areas of superimposed interlobular and intralobular septal thickening, a pattern known as crazy paving (left, box). A subsequent transbronchial lung biopsy showed multiple noncaseating granulomas (middle, inset showing granuloma; hematoxylin and eosin stain). Bronchoalveolar lavage cultures, histopathological analysis, and molecular testing were negative for infectious organisms, including Mycobacterium tuberculosis. Which of the following is the most likely diagnosis?

一名28岁女性因干咳6个月来院就诊。患者不吸烟，自诉无发热、关节痛、眼痛或皮疹。体格检查时，双下肺听诊显示细微的爆裂声。胸部高分辨率计算机断层扫描(CT)显示纵隔淋巴结肿大和弥漫性磨玻璃影(左侧，轴位)。也可以看到小叶间和小叶内间隔重叠增厚的区域，这种模式被称为“铺路石”(左，方框)。随后的经支气管肺活检显示多个非干酪性肉芽肿(图中显示肉芽肿;苏木精-伊红染色)。支气管肺泡灌洗液培养、组织病理学分析和分子检测结果均为结核分枝杆菌等感染性微生物阴性。下列哪一项是最可能的诊断?

A. Foreign body granulomatosis 异物肉芽肿病

B. Granulomatosis with polyangiitis 肉芽肿性多血管炎

C. Pulmonary alveolar proteinosis 肺泡蛋白沉积症

D. Pulmonary Langerhans cell histiocytosis 肺朗格汉斯细胞组织细胞增生症

E. Pulmonary Sarcoidosis肺结节病

答案解析：

诊断为肺结节病。“铺路石征”通常与肺泡蛋白沉积症相关，但也可见于多种其他疾病，如急性呼吸窘迫综合征、耶氏肺孢子菌肺炎和结节病(如此例)。开始逐渐减少泼尼松剂量。治疗开始后6个月，患者的症状减轻，影像学检查结果大大改善(右)。

正确答案：E