Kristin J, Beutner C, Klenzner T, Knapp F B
B-ENT. 2014;10(3):231-5.
Granulomatosis with polyangiitis is characterized by vasculitis of small and medium sized vessels and non-caseating granulomas with head and neck symptoms in 95% of those affected. Cranial nerve palsies are rare; while, chronic rhinosinusitis and ear problems are common.
We describe the serious course and the diagnostic challenge of a patient with granulomatosis with polyangiitis of bilateral mastoids and the right temporal lobe. Initially, the patient showed metachronous bilateral facial palsy with chronic mastoiditis. Repeated surgeries and rheumatologic examinations did not determine a diagnosis. The patient developed additional cranial nerve palsies. Due to progression into the temporal lobe, we removed the affected parts. After 6 months, the diagnosis was revealed by histology.
Granulomatosis with polyangiitis is a diagnostic challenge. Persistent reevaluations were necessary for a final diagnosis and to limit the life-threatening disease. Once diagnosed, therapy began with the standard FAUCI-Scheme.
肉芽肿性多血管炎的特征是中小血管血管炎以及非干酪样肉芽肿，95%的患者有头颈部症状。颅神经麻痹罕见，而慢性鼻窦炎和耳部问题常见。
我们描述了一名双侧乳突和右侧颞叶肉芽肿性多血管炎患者的严重病程及诊断挑战。最初，患者表现为异时性双侧面神经麻痹伴慢性乳突炎。反复手术和风湿科检查均未明确诊断。患者又出现了其他颅神经麻痹。由于病情进展至颞叶，我们切除了受累部位。6个月后，组织学检查确诊。
肉芽肿性多血管炎是一项诊断挑战。为最终确诊并控制这种危及生命的疾病，持续重新评估很有必要。一旦确诊，即开始采用标准的福西方案进行治疗。
