Este protocolo aborda la glomerulonefritis rápidamente progresiva (GNRP) en pacientes ANCA-positivos — una presentación frecuente con una base de evidencia relativamente sólida. La GNRP pauci-inmune ANCA-negativa se enmarca dentro del mismo contexto clínico.
DOI: 10.1007/s10157-015-1218-8
These guidelines focus on ANCA-positive RPGN, which appears frequently and for which there is relatively strong evidence, and on addressing the severe primary diseases, namely lupus nephritis and anti-GBM antibody RPGN.
For ANCA-negative pauci-immune RPGN, we recommend that the treatment be similar to that of ANCA-positive disease.
As the initial therapy for ANCA-positive RPGN, addition of rituximab to corticosteroids may improve renal and patient survival. Therefore, rituximab is recommended in cases in which standard therapy cannot be given because of adverse effects, or in those who are refractory to or relapsed after standard therapy (insurance is applicable only for patients with MPA and GPA in Japan).
Although there is limited evidence showing that IVIg improves renal and patient survival in RPGN, IVIg can be used as an alternative option for patients with refractory ANCA-associated vasculitis or those with concurrent complications such as severe infections when it is advisable to avoid the standard therapy with high-dose steroids and immunosuppressant (off-label use).
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