Costa Carlos, Santiago Tânia, Espirito-Santo Joana, Rovisco João, Silva Jorge, Malcata Armando
Centro Hospitalar e Universitário de Coimbra.
Acta Reumatol Port. 2017 Jan-Mar;42(1):82-87.
Granulomatosis with polyangiitis (GPA) is a rare autoimmune disease characterized by granulomatous inflammation involving upper and lower respiratory tract, kidneys and peripheral nervous system. However, central nervous system involvement is uncommon and frequently refractory to classical therapy. Rituximab has emerged as promising alternative, but published reports are scarce. We report a case of pachymeningitis and cerebral granuloma in a patient with a history of severe generalized GPA, treated with rituximab. This case illustrates the complexity of the management of neurologic manifestations and provides insight into the potential utility of rituximab in this condition.
肉芽肿性多血管炎(GPA)是一种罕见的自身免疫性疾病,其特征为肉芽肿性炎症累及上、下呼吸道、肾脏和周围神经系统。然而,中枢神经系统受累并不常见,且对传统治疗往往耐药。利妥昔单抗已成为一种有前景的替代治疗药物,但相关报道较少。我们报告1例有严重全身性GPA病史的患者发生硬脑膜炎和脑肉芽肿,接受了利妥昔单抗治疗。该病例说明了神经系统表现管理的复杂性,并为利妥昔单抗在这种情况下的潜在应用提供了见解。