University of Alabama, Medical Center, Birmingham, AL, United States.
University of Alabama, Medical Center, Birmingham, AL, United States.
Am J Med Sci. 2022 Oct;364(4):466-471. doi: 10.1016/j.amjms.2022.04.025. Epub 2022 Apr 29.
Necrotizing sarcoid granulomatosis (NSG) is a rare disease that shares similarities with pulmonary vasculitides and sarcoidosis. This is a report of two cases of NSG with a review of literature. The first case is a 33-year-old black female with a one-year history of malaise and cough. Lung imaging revealed scattered pulmonary nodules. Histopathology showed multiple necrotizing granulomas without prominent neutrophilic infiltrates. The second case is a 58-year-old white female with a one-year history of fatigue, dyspnea, and ophthalmoplegia on the left eye. Imaging showed multiple pulmonary nodules. Lung biopsy was consistent with NSG. The challenge of the NSG diagnosis is to distinguish it from other mimickers. Pathology often shows necrotizing granulomatous vasculitis, distinguishing it from classical sarcoid. Laboratory markers for vasculitis like neutrophil cytoplasmic antibodies and antibodies against myeloperoxidase and proteinase 3 are negative or only low titers. NSG responds well to immune-suppression, most commonly with glucocorticoids.
坏死性结节病性肉芽肿病 (NSG) 是一种罕见疾病,与肺血管炎和结节病有相似之处。这是两例 NSG 的报告,并进行了文献复习。第一个病例是一名 33 岁的黑人女性,有一年的不适和咳嗽病史。肺部影像学显示散在的肺结节。组织病理学显示多发性坏死性肉芽肿,无明显中性粒细胞浸润。第二个病例是一名 58 岁的白人女性,有一年的疲劳、呼吸困难和左眼动眼神经麻痹病史。影像学显示多发性肺结节。肺活检符合 NSG。NSG 诊断的挑战在于将其与其他类似疾病区分开来。病理学通常表现为坏死性肉芽肿性血管炎,与经典的结节病不同。血管炎的实验室标志物,如中性粒细胞胞质抗体和针对髓过氧化物酶和蛋白酶 3 的抗体,为阴性或仅低滴度。NSG 对免疫抑制反应良好,最常用的是糖皮质激素。