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一名普通可变免疫缺陷患者的肉芽肿性淋巴细胞间质性肺病

Granulomatous-Lymphocytic Interstitial Lung Disease in a Patient With Common Variable Immunodeficiency.

作者信息

Shah Jehan L, Amin Sagar B, Verma Nupur, Mohammed Tan-Lucien

机构信息

Department of Radiology, University of Florida, Gainesville, FL.

Department of Radiology, University of Florida, Gainesville, FL.

出版信息

Curr Probl Diagn Radiol. 2018 Jul-Aug;47(4):282-284. doi: 10.1067/j.cpradiol.2017.04.007. Epub 2017 Apr 14.

Abstract

Common variable immunodeficiency is the most common primary immunodeficiency and consists of impaired immunoglobulin production causing recurrent sinopulmonary infections. The most common cause of mortality for this disorder, however, is from the development of malignancy and autoimmune disorders. One common entity that develops is a systemic granulomatous and lymphoproliferative disorder that can cause an interstitial lung disease more formally referred to as granulomatous-lymphocytic interstitial lung disease (GL-ILD). We discuss a case of a 25-year-old woman with common variable immunodeficiency and GL-ILD and review the literature to summarize the most common radiological findings to raise the suspicion for GL-ILD on high-resolution computed tomography and delineate this from infection and other mimickers. We will also review key histopathological characteristics for diagnosis and the clinical approach and treatment options for this rare disease.

摘要

普通可变免疫缺陷是最常见的原发性免疫缺陷,由免疫球蛋白产生受损导致反复的鼻窦肺部感染组成。然而,这种疾病最常见的死亡原因是恶性肿瘤和自身免疫性疾病的发生。一种常见的疾病是系统性肉芽肿性和淋巴细胞增殖性疾病,可导致间质性肺病,更正式地称为肉芽肿性淋巴细胞间质性肺病(GL-ILD)。我们讨论了一例25岁患有普通可变免疫缺陷和GL-ILD的女性病例,并回顾文献以总结最常见的影像学表现,以提高在高分辨率计算机断层扫描上对GL-ILD的怀疑,并将其与感染和其他类似疾病区分开来。我们还将回顾诊断的关键组织病理学特征以及这种罕见疾病的临床方法和治疗选择。

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