Rapidly progressive glomerulonephritis (RPGN) presenting in the context of lupus nephritis — particularly diffuse proliferative disease (class IV, and select class III cases) — represents one of the most acute and severe renal manifestations of systemic lupus erythematosus. Early, protocol-guided management is essential to protect renal function and improve survival.
This protocol addresses patients with lupus nephritis (diffuse proliferative, class IV and some class III) who present with RPGN. In this sub-population, the combination of severe histological injury and rapidly declining renal function demands a structured therapeutic approach. Evidence shows that adding immunosuppressive agents to corticosteroids in the initial therapy has been associated with improved renal function and survival in these patients.
When standard treatment options are unavailable, a biologic agent targeting B cells may be considered as part of the management strategy. The complete regimen — including specific agents, sequencing, and criteria for use — is available in the full protocol.
We recommend immunosuppressive agents (cyclophosphamide or mycophenolate mofetil) with corticosteroids as the initial therapy for patients with diffuse proliferative lupus nephritis.
In patients with lupus nephritis presenting with RPGN (class IV and some class III cases), the addition of immunosuppressive agents to corticosteroids in the initial therapy has been shown to improve renal function and survival.
In patients with lupus nephritis presenting with RPGN (class IV and some class III cases), there is no evidence to support that treatment with rituximab improves renal function and survival; however, it could be considered if there is no other treatment available (not covered by insurance in Japan).
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