肺间质性疾病表现为双侧肺结节

F. Mfone, O. Enilari, V. Pathak
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引用次数: 0

摘要

简介:间质性肺疾病(ILD)是一组异质性疾病,具有相似的临床、放射学和生理学表现,因此大多归为一类。放射学上,他们表现出不同的表现,包括磨砂玻璃影、网状、蜂蜜梳状、间隔增厚或这些表现的组合。如果放射学表现与临床表现相吻合,某些特定的组合可能导致特定ILD的诊断。我们报告一位慢性咳嗽及呼吸短促的病人,胸部CT显示前纵隔肿块伴双侧肺结节。手术肺活检显示细胞性非特异性间质性肺炎NSIP和淋巴样间质性肺炎LIP。病例:一名30岁女性,因进行性呼吸困难入院约3周,病情恶化,并伴有运动耐量下降。所有的常规实验室检查都很正常。炎症标志物如CRP、ESR、LDH均略有升高。新冠病毒检测呈阴性。胸部CT血管造影显示右上肺叶前部8厘米肿块,双侧肺野可见大量肺结节。脑核磁共振、骨扫描和腹部和骨盆CT扫描未显示任何转移性疾病的证据。ct引导下对肺前部肿块进行了芯针活检,并进行了几次风湿病血液检查。抗核抗体阳性,RNP抗体3.2升高(文献<1), SS-A/Ro自身抗体6.5升高(文献<1)。肺前部肿块的核心穿刺病理为胸腺瘤阳性。进行了视频辅助胸腔镜肺手术。肺结节病理表现为细胞性非特异性间质性肺炎与LIP重叠。对干燥相关的间质性肺疾病和胸腺瘤进行诊断。给予麦考酚酸盐和强的松治疗。讨论:NSIP主要是特发性的,但它可以被发现与结缔组织疾病相关,包括干燥病、狼疮、皮肌炎和多发性肌炎。NSIP的CT表现常为牵引性支气管扩张伴对称及双侧磨玻璃影,无蜂窝状。上述病例描述了一名双侧肺结节患者,最初被认为是前胸壁肿块的转移。虽然被认为非常罕见,但与结缔组织疾病相关的前胸壁肿块包括胸腺瘤、胸腺囊肿、畸胎瘤和胸腺癌。我们的病人有罕见的干燥相关性间质性肺疾病和胸腺瘤,并有非典型的NSIP CT表现。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Interstitial Lung Disease Presenting with Bilateral Pulmonary Nodules
Introduction: Interstitial Lung Disease (ILD) is the heterogenous group of disease that share similar clinical, radiological and physiological findings and hence mostly grouped together. Radiologically, they present with varied findings which includes ground glass opacity, reticulations, honey combing, septal thickenings or the combination of these findings. Some specific combination could lead to the diagnosis of a particular ILD provided radiological findings match the clinical presentations. We present a patient who came with chronic cough and shortness of breath and CT chest showed anterior mediastinal mass with bilateral pulmonary nodules. Surgical lung biopsy revealed cellular NSIP (nonspecific interstitial pneumonia) and LIP (lymphoid interstitial pneumonia). Case: A 30-year-old female presented to the hospital with progressive dyspnea for approximately 3 weeks that had worsened and was associated with decreased exercise tolerance. All routine laboratory workup was unremarkable. Inflammatory markers like CRP, ESR, LDH were slightly elevated. COVID-19 test was negative. A CT angiography of the thorax demonstrated an 8cm anterior right upper lobe pulmonary mass with numerous pulmonary nodules in bilateral lung fields. MRI of the brain, bone scan and CT scan of the abdomen and pelvis did not demonstrate any evidence of metastatic disease. A CT-guided core needle biopsy of the anterior lung mass was performed as well as several rheumatologic blood tests. Antinuclear antibody was positive with elevated RNP antibody 3.2 (reference <1), elevated autoantibodies to SS-A/Ro 6.5 (reference <1). The pathology of the core needle biopsy of the anterior lung mass was positive for thymoma. A video assisted thoracoscopic surgery of the lung was performed. The pathology of the pulmonary nodules was positive for overlapping features of cellular nonspecific interstitial pneumonia and LIP. The diagnoses of Sjogren's related interstitial lung disease and thymoma were made. She was treated with mycophenolate and prednisone. Discussion: NSIP is primarily idiopathic however it can be found in association with connective tissue disorders including Sjogren's disease, lupus, dermatomyositis, and polymyositis. CT findings for NSIP often show traction bronchiectasis with symmetric and bilateral ground-glass opacities with no honeycombing. This case above describes a patient with bilateral pulmonary nodules that were initially thought to be metastatic from her accompanying anterior chest wall mass. Although considered very rare, anterior chest wall masses that are associated with connective tissue diseases include thymoma, thymic cyst, teratoma, and thymic carcinoma. Our patient had a rare presentation of Sjogren associated interstitial lung disease and thymoma with atypical CT findings of NSIP.
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