Department of Internal Medicine, Seoul National University Hospital, Seoul, Korea.
J Korean Med Sci. 2012 Mar;27(3):317-20. doi: 10.3346/jkms.2012.27.3.317. Epub 2012 Feb 23.
During the last five decades, long-term therapy with immunosuppressive agents such as pulse cyclophosphamide in conjunction with high-dose corticosteroids has enhanced both patient survival and renal survival in patients with diffuse proliferative lupus nephritis. Nevertheless, severe side effects such as infectious complications remain the main cause of morbidity and mortality. Central nervous system aspergillosis is uncommon but life-threatening in lupus patients. In this single-patient case study, carotid aneurysm with sphenoidal sinusitis was suspected when severe epistaxis occurred during cyclophosphamide pulse therapy. With anti-fungal therapy, a graft stent was successfully deployed to the aneurysm and specimens of sphenoidal mucosa showed typical hyphae, indicating aspergillosis. Three months after stopping voriconazole treatment, two cerebral aneurysms that were revealed on MR images were successfully removed by aneurysmal clipping. The patient remained alive at one-year follow-up with lupus nephritis in remission. The rarity and high mortality of aspergillus-related fungal aneurysms have led to most cases being recognized postmortem. However, such aneurysms must be diagnosed early to prevent fatal complications by performing appropriate management such as surgical procedure or endovascular intervention.
在过去的五十年中,长期使用免疫抑制剂治疗,如脉冲环磷酰胺联合大剂量皮质类固醇,提高了弥漫性增生性狼疮肾炎患者的生存率和肾脏生存率。然而,严重的副作用,如感染并发症仍然是发病率和死亡率的主要原因。中枢神经系统曲霉菌病在狼疮患者中并不常见,但却有生命危险。在这例单病例研究中,当在环磷酰胺脉冲治疗期间发生严重鼻出血时,怀疑存在颈动脉动脉瘤伴蝶窦炎。经过抗真菌治疗,成功地将移植物支架放置在动脉瘤处,蝶窦黏膜标本显示出典型的菌丝,提示为曲霉菌病。停用伏立康唑治疗三个月后,磁共振成像显示的两个脑动脉瘤通过动脉瘤夹闭成功切除。患者在狼疮肾炎缓解的一年随访中仍然存活。由于与曲霉菌相关的真菌性动脉瘤的罕见性和高死亡率,导致大多数病例都是在死后才被发现。然而,必须通过适当的管理,如手术或血管内介入,来早期诊断这些动脉瘤,以防止致命的并发症。