Department of Internal Medicine, Ghent University Hospital, Ghent, Belgium.
Department of Pathological Anatomy, Ghent University Hospital, Ghent, Belgium.
Acta Clin Belg. 2021 Oct;76(5):384-391. doi: 10.1080/17843286.2020.1740858. Epub 2020 Mar 13.
: Guillain-Barré Syndrome usually presents with ascending symmetric polyneuropathy, typically preceded by a viral infection. Despite the low incidence, physicians will often include Guillain-Barré Syndrome in their differential diagnosis. However, another underlying cause of polyneuropathy known as ANCA-associated vasculitis (AAV) is even more rare than Guillain-Barré Syndrome and therefore is usually overlooked. AAV has a broad spectrum of symptomatology and sometimes presents only with neurological complaints. If treated inappropriately, AAV can be lethal.: In this case report, we describe a 72-year-old man presenting with complaints of symmetric polyneuropathy and paresis of both legs, initially diagnosed as Guillain-Barré Syndrome. During his treatment with intravenous immunoglobulins, he developed acute renal failure. Further investigations showed ANCA positive pauci-immune acute glomerulonephritis. This, in combination with eosinophilia and sinusitis, led to a final diagnosis of Eosinophilic Granulomatosis with Polyangiitis EGPA (Churg-Strauss disease). Induction therapy was initiated using glucocorticoids, cyclophosphamide and temporary plasmapheresis, followed by maintenance therapy with azathioprine complicated by bone marrow suppression. Azathioprine was discontinued and monotherapy with low-dose glucocorticoids was continued with the recovery of bone marrow function, good clinical condition and no relapse of vasculitis at 14 months follow-up.: Physicians should be aware of the possible presentations of AAV. When suspected, indirect immunofluorescence assay for ANCA should be performed. When AAV is diagnosed, induction therapy should be administered as soon as possible, followed by maintenance therapy and careful follow-up, as patients are at risk for opportunistic infections, bone marrow toxicity or relapse.
格林-巴利综合征通常表现为上升性对称性多发性神经病,通常在病毒感染之前发生。尽管发病率较低,但医生通常会将格林-巴利综合征纳入鉴别诊断。然而,另一种称为抗中性粒细胞胞质抗体(ANCA)相关性血管炎(AAV)的潜在原因更为罕见,因此通常被忽视。AAV 具有广泛的症状谱,有时仅表现为神经系统症状。如果治疗不当,AAV 可能是致命的。
在本病例报告中,我们描述了一名 72 岁男性,他因对称性多发性神经病和双腿无力就诊,最初被诊断为格林-巴利综合征。在接受静脉注射免疫球蛋白治疗期间,他出现急性肾衰竭。进一步的检查显示 ANCA 阳性少免疫性急性肾小球肾炎。这与嗜酸性粒细胞增多和鼻窦炎一起,导致最终诊断为嗜酸性肉芽肿伴多血管炎(EGPA)(Churg-Strauss 病)。使用糖皮质激素、环磷酰胺和临时血浆置换开始诱导治疗,随后用硫唑嘌呤维持治疗,同时伴有骨髓抑制。由于骨髓功能恢复、临床状况良好且血管炎无复发,停用硫唑嘌呤并继续使用低剂量糖皮质激素单药治疗,随访 14 个月。
医生应该意识到 AAV 的可能表现。当怀疑时,应进行间接免疫荧光法 ANCA 检测。一旦诊断为 AAV,应尽快进行诱导治疗,然后进行维持治疗和仔细随访,因为患者有机会感染、骨髓毒性或复发的风险。