Doença relacionada ao IGG4 com manifestação neurológica: um relato de caso e revisão da literatura

Abstract

Introduction: IgG4-related disease is a fibroinflammatory condition that can affect multiple organs, including the central nervous system. This study presents a case with severe neurological involvement and reviews the main clinical, diagnostic, and therapeutic aspects of the disease. Methodology: This study consists of a case report accompanied by a narrative literature review, conducted in international databases using descriptors related to IgG4-related disease and neurological involvement. Case Report: A 60-year-old male patient presented with frontal headache and progressive visual loss evolving to bilateral blindness. Brain MRI revealed diffuse pachymeningitis, and serum IgG4 levels were elevated. The patient showed limited response to pulse therapy, prednisone, and azathioprine, and rituximab was indicated as maintenance therapy. Discussion: IgG4-related pachymeningitis can lead to rapid visual decline, requiring early diagnosis. The variable response to corticosteroids and conventional immunosuppressants highlights the relevance of rituximab in refractory cases. Conclusion: This case illustrates the diagnostic and therapeutic challenges of IgG4-related disease with neurological involvement. Early identification and access to effective therapies are essential to prevent permanent sequelae, emphasizing the need for a multidisciplinary approach.