Rapidly Progressive Glomerulonephritis
ICD-10 N01.7 · ICD-11 GB40.2

Treatment of Rapidly Progressive Glomerulonephritis in ANCA-Positive Disease

This protocol covers rapidly progressive glomerulonephritis (RPGN) in ANCA-positive patients — a frequently encountered presentation with a relatively strong evidence base. ANCA-negative pauci-immune RPGN falls within the same clinical framework.

Clinical scenario ANCA-positive rapidly progressive glomerulonephritis is a focus of these guidelines because of its frequency and the strength of available evidence. ANCA-negative pauci-immune RPGN is treated along the same lines.
Treatment approach (partial overview) The approach centres on targeted biological immunotherapy combined with corticosteroids, applied when standard therapy is not feasible due to adverse effects or when the patient has not responded to or has relapsed following standard treatment. A separate immunomodulatory option is considered in refractory settings or when concurrent clinical factors make standard therapy inadvisable.

The full protocol — including sequencing, specific indications, decision points, and alternative strategies — is available via the link below.

Instant Access to Structured Evidence-Based Regimens

References

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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