Publicação
IgG4-tubulointerstitial nephritis: a literature review following 5 cases in a single centre
| Resumo: | Immunoglobulin G4-related disease is a recently described systemic fibro-inflammatory disease, characterized by an infiltration of abundant IgG4+ plasma cells and lymphocytes, leading to tumour-like swellings of the involved organs, and variable degrees of fibrosis. Organ involvement can occur metachronously, and as IgG4 expressionin other tissues is less specific, renal biopsy often plays a critical role in diagnosis leading to prompt treatment, which can prevent renal damage and other organ failure. Tubulointerstitial nephritis is the most common renal feature. IgG4-renal involvement is challenging to diagnose and remains under-recognized, particularly in patients with single-organ involvement. The authors describe the only 5 cases of IgG4-RD with kidney involvement identified in the last 10 years in a tertiary referral nephrology centre that serves a population of 3 million people. These cases underline the importance of an early diagnosis of IgG4-related disease, since early treatment is usually effective. They also corroborate that clinical and histopathological features are heterogeneous and that complement may play a role in its pathogenesis |
|---|---|
| Autores principais: | Rocha,Joana |
| Outros Autores: | Kang,Amy; Wong,Nikki; Westaby,Joseph; Roufosse,Candice; Cook,Terence |
| Assunto: | IgG4 tubulo-interstitial nephritis |
| Ano: | 2017 |
| País: | Portugal |
| Tipo de documento: | artigo |
| Tipo de acesso: | acesso aberto |
| Instituição associada: | Fundação para a Ciência e Tecnologia |
| Idioma: | inglês |
| Origem: | SciELO Portugal |
| Resumo: | Immunoglobulin G4-related disease is a recently described systemic fibro-inflammatory disease, characterized by an infiltration of abundant IgG4+ plasma cells and lymphocytes, leading to tumour-like swellings of the involved organs, and variable degrees of fibrosis. Organ involvement can occur metachronously, and as IgG4 expressionin other tissues is less specific, renal biopsy often plays a critical role in diagnosis leading to prompt treatment, which can prevent renal damage and other organ failure. Tubulointerstitial nephritis is the most common renal feature. IgG4-renal involvement is challenging to diagnose and remains under-recognized, particularly in patients with single-organ involvement. The authors describe the only 5 cases of IgG4-RD with kidney involvement identified in the last 10 years in a tertiary referral nephrology centre that serves a population of 3 million people. These cases underline the importance of an early diagnosis of IgG4-related disease, since early treatment is usually effective. They also corroborate that clinical and histopathological features are heterogeneous and that complement may play a role in its pathogenesis |
|---|