From the Guidelines
Crescentic glomerulonephritis should be treated with high-dose glucocorticoids and cyclophosphamide immediately, as recommended by the most recent guideline evidence 1. This approach is crucial in managing the disease, which is characterized by the formation of crescent-shaped structures in the glomeruli, leading to rapidly progressive kidney disease. The treatment regimen should include:
- High-dose corticosteroids, typically methylprednisolone 500-1000mg intravenously daily for 3 days, followed by oral prednisone 1mg/kg/day
- Cyclophosphamide, either 2mg/kg/day orally or 15mg/kg intravenously every 2-3 weeks Additional considerations include:
- Plasma exchange for anti-GBM disease, performed daily for 14 days or until antibodies are undetectable
- Rituximab (375mg/m² weekly for 4 weeks) as an alternative to cyclophosphamide for ANCA-associated vasculitis Maintenance therapy typically involves azathioprine (2mg/kg/day) or mycophenolate mofetil (1-2g/day) for 18-24 months after remission, with supportive care including blood pressure control, dialysis if needed, and prevention of treatment complications 1. Early diagnosis and aggressive immunosuppression are essential to prevent rapid progression to kidney failure, as the pathophysiology involves immune complex deposition or autoantibodies targeting the glomerular basement membrane or neutrophil components, triggering inflammation that damages the glomeruli and forms the characteristic crescents. Key points to consider in the management of crescentic glomerulonephritis include:
- The intensity of induction therapy is predicated on the severity of presenting symptoms and type of glomerulonephritis
- The level of GFR needs to be taken into account for determining safe dosage
- Proteinuria reduction is a surrogate endpoint in the treatment of glomerulonephritis
- Maintenance therapy with an immunosuppressive is not required for patients who recover from anti-GBM disease, unless they are also positive for ANCA.
From the Research
Definition and Classification of Crescentic Glomerulonephritis
- Crescentic glomerulonephritis (GN) is a life-threatening clinical syndrome characterized by crescents, which are morphological manifestations of severe glomerular injury 2.
- Immunologically, crescentic GN can be classified into anti-glomerular basement membrane nephritis, immune complex-mediated GN, and pauci-immune GN 2, 3.
Treatment Options for Crescentic Glomerulonephritis
- Conventional treatment for crescentic GN mainly consists of corticosteroids, immunosuppressants, and plasma exchange 2.
- Plasmapheresis is beneficial for patients with Goodpasture's syndrome and those with severe pauci-immune GN 2, 4.
- New therapeutic agents, such as monoclonal antibodies to T cells, B cells, and cytokines, and signal transduction inhibitors, may provide satisfactory alternatives 2.
- Treatment for anti-GBM antibody-induced crescentic GN should include pulse methylprednisolone, plasmapheresis, and corticosteroids and cyclophosphamide 4.
- Treatment for pauci-immune crescentic GN should include pulse methylprednisolone, oral corticosteroids, and cyclophosphamide 4.
Prognosis and Outcome of Crescentic Glomerulonephritis
- The prognosis of crescentic GN has improved in recent decades, but early aggressive therapy is still recommended due to the high risk of end-stage renal disease (ESRD) 4.
- Patient outcomes can vary depending on the underlying disease, duration of disease, percentage of crescentic glomeruli, and treatment response 5.
- Early diagnosis and aggressive therapy are critical to prevent progression to ESRD 3, 5.
Emerging Therapies for Crescentic Glomerulonephritis
- Human amniotic epithelial cells (hAECs) represent a novel therapeutic option for the treatment of crescentic GN due to their unique immunosuppressive ability and excellent safety profile 6.
- Clinical trials are needed to establish the safety and efficacy of new therapeutic agents for crescentic GN 2, 3.