This protocol addresses ANCA-positive rapidly progressive glomerulonephritis (RPGN) — a presentation for which there is relatively strong evidence. ANCA-negative pauci-immune RPGN is treated identically and falls within the same guideline framework.
The guideline places particular emphasis on presentations complicated by advanced renal dysfunction or diffuse alveolar hemorrhage. These high-risk features determine whether an additional intervention is appended to the immunosuppressive foundation.
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.
In patients with ANCA-positive RPGN complicated with advanced renal dysfunction or pulmonary hemorrhage, the addition of plasmapheresis to immunosuppressive therapy as the initial therapy may improve renal function and survival. We recommend the addition of plasmapheresis in such patients.
The addition of plasmapheresis to the initial therapy with corticosteroids and cyclophosphamide is indicated for patients presenting with advanced kidney failure (serum creatinine, [5.8 mg/dL) or those with diffuse alveolar hemorrhage.
View source ↗