Doença Pulmonar Intersticial Linfocítica Granulomatosa (GLILD)
Granulomatous Lymphocytic Interstitial Lung Disease (GLILD)
Joseane Chiabai; Thais Costa Lima de Moura; Mayra de Barros Dorna; Gesmar; Rodrigues Silva Segundo
Resumo
A Doença Pulmonar Intersticial Linfocítica Granulomatosa (GLILD) é uma das complicações graves dos erros inatos da imunidade e está relacionada à desregulação imunológica e a fenômenos autoimunes. O diagnóstico requer associação das manifestações clínicas aos achados funcionais, tomográficos e histológicos. O tratamento imunossupressor, com corticosteroides ou agentes citotóxicos, é a primeira linha de abordagem, visando controlar a inflamação. No entanto, a resposta à terapia é variável e muitos pacientes apresentam progressão da doença e desenvolvimento de fibrose pulmonar. Nesta revisão destacamos os aspectos mais relevantes sobre a patogênese e diagnóstico, discutindo-se as opções terapêuticas, com base nas publicações dos últimos 10 anos.
Palavras-chave
Abstract
Granulomatous lymphocytic interstitial lung disease (GLILD) is a serious complication of inborn errors of immunity and is associated with immune dysregulation and autoimmune phenomena. Diagnosis requires the integration of clinical manifestations with functional, tomographic, and histological findings. Immunosuppressive therapy, with corticosteroids or cytotoxic agents, is the first-line approach aimed at controlling inflammation. However, response to therapy is variable, and many patients experience disease progression with the development of pulmonary fibrosis. This review highlights the most relevant aspects of GLILD pathogenesis and diagnosis and discusses treatment options, based on publications from the past 10 years
Keywords
Referências
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Submetido em:
16/02/2025
Aceito em:
07/08/2025
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