■坏死生物黄色肉芽肿(NXG)是一种非朗格汉斯细胞组织细胞增生症和多系统疾病。低水平的HDL胆固醇与全身性炎症相关,这可能是单克隆免疫球蛋白和脂蛋白相互作用的结果,是一个特征。没有证据表明NXG相关的大血管血管炎,也没有任何既定的治疗方法,尽管合并多发性骨髓瘤的化疗是最常见的。
■我们描述了一个53岁男性的病例,该病例首次出现心力衰竭,收缩功能受损。他出现了眼眶黄色瘤和多个皮下结节,实验室检查显示C反应蛋白水平升高,低HDL,和副蛋白血症.这些临床特征和皮肤活检的病理发现导致了NXG的诊断。18F-氟脱氧葡萄糖正电子发射断层扫描(PET)/计算机断层扫描(CT)证实主动脉和双侧颈总动脉的摄取增加。他开始使用泼尼松龙治疗大血管血管炎。治疗后,随着载脂蛋白A1(Apo-A1)和HDL水平的显着升高,C反应蛋白立即降低。6个月随访时收缩功能障碍恢复。患者治疗后5年未出现心力衰竭,随访PET/CT显示血管炎症的分辨率。
■这是NXG相关大血管血管炎的第一份报告。低剂量泼尼松龙可能对NXG相关血管炎和心肌病有益。HDL,Apo-A1和C反应蛋白水平可能有助于监测NXG的活性,PET/CT是NXG相关性血管炎的有价值的诊断工具。
UNASSIGNED: Necrobiotic xanthogranuloma (NXG) is a non-Langerhans cell histiocytosis and multisystem disorder. Low level of HDL cholesterol associated with a systemic inflammatory profile, which may result from the interaction of monoclonal immunoglobulin and lipoproteins, is a characteristic feature. There is no evidence of NXG-associated large-vessel vasculitis, nor are there any established treatments, although chemotherapy for comorbid multiple myeloma is most often administered.
UNASSIGNED: We describe a case of a 53-year-old male with a first history of heart failure with impaired systolic function. He presented with orbital xanthomas and multiple subcutaneous nodules, and laboratory examination showed elevated levels of C-reactive protein, low HDL, and paraproteinemia. A constellation of these clinical features and pathological findings of skin biopsy led to the diagnosis of NXG. 18F-Fluorodeoxyglucose positron emission tomography (PET)/computed tomography (CT) confirmed increased uptake in the aorta and bilateral common carotid arteries. He began prednisolone treatment with reference to treatment for large-vessel vasculitis. After the treatment, C-reactive protein immediately decreased with markedly increased levels of apolipoprotein A1 (Apo-A1) and HDL. Systolic dysfunction was restored at 6-month follow-up. The patient has not experienced heart failure for 5 years after treatment, and the follow-up PET/CT demonstrated resolution of vascular inflammation.
UNASSIGNED: This is the first report of NXG-associated large-vessel vasculitis. Low-dose prednisolone may benefit for NXG-associated vasculitis and cardiomyopathy. HDL, Apo-A1, and C-reactive protein levels may be useful for monitoring the activity of NXG, and PET/CT was a valuable diagnostic tool for NXG-associated vasculitis.