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Características clínicas e padrões de terapêutica em doentes com glomerulopatias primárias
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Introdução: As glomerulopatias são responsáveis por cerca de 20% dos casos de DRC globalmente. O tratamento adequado é essencial para diminuir a progressão da doença. Objetivos: Descrever as caraterísticas demográficas, clínicas e laboratoriais dos doentes com DLM, GESF, N-IgA e NM; descrever as abordagens terapêuticas atuais e avaliar a resposta à terapêutica.
Métodos: Realizámos um estudo retrospetivo unicêntrico de doentes adultos com DLM, GESF, N-IgA ou NM seguidos no CHULN durante 2019. Foram excluídos doentes com DRC sob terapia de substituição renal e doentes perdidos no follow-up. Resultados: Identificámos 159 doentes, 44.0% com N-IgA, 24.5% com GESF, 17.0% com DLM e 14.5% com NM. Na DLM todos os doentes realizaram terapêutica de 1ª linha com CCT, e 62.5% dos que transitaram para 2ª linha foram tratados com inibidores da calcineurina. Na GESF primária a CCT foi a 1ª linha mais usada, a 2ª linha foi necessária em 53.8% doentes, sendo a ciclosporina o fármaco mais usado e como 3ª linha os fármacos mais usados foram o tacrolimus, o RTX e o MMF. Na N-IgA 75.7% dos doentes realizaram apenas tratamento conservador. Dos que iniciaram IS, 82.3% foram tratados com CCT e 58.8% obtiveram remissão. Na NM 71.4% dos doentes cumpriram 6 meses de tratamento conservador. A maioria dos doentes realizou IS, dos quais 64.3% foram tratados com inibidores da calcineurina. O RTX foi o fármaco de 2ª e 3ª linha mais utilizado. No último follow-up, globalmente verificou-se diminuição da ProtU média e ligeiro agravamento da função renal. Conclusões: Neste estudo identificámos discordância entre a prática clínica atual e as guidelines da KDIGO, o que reflete os estudos mais recentes e suporta a necessidade de atualização destas guidelines.
Introduction: Glomerulonephritis are responsible for about 20% of CKD worldwide. Adequate treatment is essential to reduce disease progression. Objectives: To describe demographic, clinical and laboratorial characteristics of patients with MCD, FSGS, IgAN and MN; to describe current treatment approaches and to evaluate treatment response. Methods: We conducted a unicentric retrospective study of adult patients with MCD, FSGS, IgAN and MN followed at CHULN in 2019. Patients with CKD under renal replacement therapy and patients lost at follow-up were excluded. Results: We identified 159 patients, 44.0% with N-IgA, 24.5% with GESF, 17.0% with DLM and 14.5% with NM. In MCD, all patients underwent 1st line therapy with CCT, and 62.5% of those who transitioned to a 2nd line were treated with calcineurin inhibitors. In primary GESF CCT was the most used 1st line treatment, and 2nd line was necessary in 53.8% of patients, cyclosporine being the most used. As a 3rd line, the most used drugs were tacrolimus, RTX and MMF. In patients with IgAN, 75.7% underwent only conservative treatment. Of those who initiated immunosuppression, 82.3% were treated with CCT and 58.8% obtained remission. In MN, 71.4% of patients fulfilled 6 months of conservative treatment. Most of the patients underwent immunosuppression, of which 64.3% were treated with calcineurin inhibitors. RTX was the most used 2nd and 3rd line drug. On the last follow-up, we observed a reduction in median proteinuria and a slight decline in renal function overall. Conclusions: In this study we identified poor alignment between current clinical practice and KDIGO guidelines, which reflects recent studies and supports the need for an update of these guidelines.
Introduction: Glomerulonephritis are responsible for about 20% of CKD worldwide. Adequate treatment is essential to reduce disease progression. Objectives: To describe demographic, clinical and laboratorial characteristics of patients with MCD, FSGS, IgAN and MN; to describe current treatment approaches and to evaluate treatment response. Methods: We conducted a unicentric retrospective study of adult patients with MCD, FSGS, IgAN and MN followed at CHULN in 2019. Patients with CKD under renal replacement therapy and patients lost at follow-up were excluded. Results: We identified 159 patients, 44.0% with N-IgA, 24.5% with GESF, 17.0% with DLM and 14.5% with NM. In MCD, all patients underwent 1st line therapy with CCT, and 62.5% of those who transitioned to a 2nd line were treated with calcineurin inhibitors. In primary GESF CCT was the most used 1st line treatment, and 2nd line was necessary in 53.8% of patients, cyclosporine being the most used. As a 3rd line, the most used drugs were tacrolimus, RTX and MMF. In patients with IgAN, 75.7% underwent only conservative treatment. Of those who initiated immunosuppression, 82.3% were treated with CCT and 58.8% obtained remission. In MN, 71.4% of patients fulfilled 6 months of conservative treatment. Most of the patients underwent immunosuppression, of which 64.3% were treated with calcineurin inhibitors. RTX was the most used 2nd and 3rd line drug. On the last follow-up, we observed a reduction in median proteinuria and a slight decline in renal function overall. Conclusions: In this study we identified poor alignment between current clinical practice and KDIGO guidelines, which reflects recent studies and supports the need for an update of these guidelines.
Descrição
Trabalho Final do Curso de Mestrado Integrado em Medicina, Faculdade de Medicina, Universidade de Lisboa, 2020
Palavras-chave
Doença de lesões mínimas Glomeruloesclerose segmentar e focal Nefropatia a IgA Nefropatia membranosa Nefrologia