Limited Antineutrophil Cytoplasmic Antibodies (ANCA)-Negative Granulomatosis With Polyangiitis: Successful Response to Rituximab

• Autor(es): Andrade, MM ; Fernandes, M ; Freire, S ; Cruz, D
• Data: 2023
• Identificador Persistente: http://hdl.handle.net/10400.26/46530
• Origem: Hospital de Cascais
• Assunto(s): Granulomatose com Poliangiite; Rituximab; Granulomatosis with Polyangiitis
• Tipo de Documento: Artigo científico
• Idioma: Inglês
• Contribuidor(es): Repositório Comum

Descrição

Granulomatosis with polyangiitis (GPA), a systemic vasculitis, is commonly characterized by the presence of antineutrophil cytoplasmic antibodies (ANCA). However, a subset of patients with limited disease may exhibit ANCA negativity. In this article, we report the case of a 40-year-old female diagnosed with GPA with intolerance to methotrexate titration and glucocorticoid therapy, leading to the initiation of rituximab treatment. Subsequently, the patient exhibited sustained clinical, laboratory, and radiological improvement. The identification of limited GPA has important therapeutic implications as the effectiveness of the medical treatment in ANCA-negative GPA may differ. Rituximab has emerged as an optimal treatment, irrespective of ANCA status, offering prolonged responses and a favorable tolerance profile in these patients.