Benucci M, Li Gobbi F, Panconesi P, Manfredi M, Sarzi-Puttini P, Atzeni F
Rheumatology Unit, San Giovanni di Dio Hospital, Florence, Italy.
Reumatismo. 2013 May 27;65(2):90-4. doi: 10.4081/reumatismo.2013.90.
Wegener's granulomatosis or granulomatosis polyangiitis associated (GPA) is a granulomatous inflammation of the upper and lower respiratory tracts associated with necrotising vasculitis of small and medium-sized blood vessels and, frequently, necrotising glomerulonephritis. We describe the case of a 37 year old female patient presenting with upper respiratory tract involvement, chronic rhinosinusitis with green secretions, and bilateral hypoacusia. Ten months later, she suffered occipital headache and two episodes of lipothymia associated with nausea, photophobia, faintness with visual blurring. Magnetic resonance imaging (MRI) revealed aseptic meningitis. The patient was treated with steroids and cyclophosphamide without any effect on the neurological symptoms which, however, greatly improved after subsequent treatment with rituximab as confirmed by means of cerebral MRI. Rituximab is an optimal means of treating cyclophosphamide-resistant patients with GPA associated with meningeal involvement.
韦格纳肉芽肿或肉芽肿性多血管炎（GPA）是一种上、下呼吸道的肉芽肿性炎症，伴有中小血管的坏死性血管炎，且常伴有坏死性肾小球肾炎。我们描述了一名37岁女性患者的病例，该患者出现上呼吸道受累、伴有绿色分泌物的慢性鼻窦炎以及双侧听力减退。10个月后，她出现枕部头痛以及两次与恶心、畏光、视物模糊伴头晕相关的晕厥发作。磁共振成像（MRI）显示为无菌性脑膜炎。该患者接受了类固醇和环磷酰胺治疗，但对神经症状没有任何效果，然而，后续使用利妥昔单抗治疗后症状有了显著改善，脑部MRI证实了这一点。利妥昔单抗是治疗伴有脑膜受累的环磷酰胺抵抗性GPA患者的一种最佳方法。
