Rapidly progressive glomerulonephritis medical therapy
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Accociate editor-in-Chief: Nazia Fuad M.D.
Overview
Rapidly progressive glomerulonephritis as obvious from its name is a syndrome that rapidly progresses to acute renal failure so early diagnosis by renal biopsy and serology is very important. The treatment should be initiated as soon as possible to minimize the irreversible renal injury. Treatment include supportive treatment and specific treatment. Supportive treatment is to treat the infection if there is one, correct the volume status with dialysis if required and smoking cessation. Specific therapy focuses on induction and maintenance of remission . Emperic therapy should be started immediatly if the disease is very severe or if renal biopsy result is delayed. After the diagnosis is established, specific therapy can be started according to subtype of the disease.
Medical therapy
Medical therapy focuses to induce and then maintain the remission.
Pharmacologic Therapy
Induction of remission
Two pharmacologic agents that are used for induction of remission in patients with rapidly progressive glomerulonephritis are;
- Corticosteroids(high dose)
- Cyclophosphamide
Glucocorticoids[1]
1. Methylprednisone
- Intravenous (IV) 7 mg/kg/d at a maximum dose of 1 g for
2. Prednisone
- 1 mg/kg/d ,PO at a maximum dose of 80 mg for 3 weeks
- 2 mg/kg/d at a maximum dose of 120 mg for 3 months.
- Taper patient off steroids by deceasing 25% of prednisone dose every 4 weeks until patient stops
Immunosuppressive Therapy
- Preffered regimen(1) : Cyclophosphamide:[2]
- Addition of cyclophosphamide is necessary to prevent relapse
- Relapse is commonly seen in patients getting glucocorticoids alone
- Can be given intraven
- IV: 15 mg/kg (maximum dose: 1,200 mg) every 2 weeks for 3 doses, followed by maintenance pulses of 15 mg/kg IV (maximum dose: 1,200 mg) every 3 weeks until after remission is achieved
- PO:1.5- 2 mg/kg/day until remission is achieved, followed by 1.5 mg/kg/day for 3 more months.
- Preffered regimen(2) Rituximab IV, 375 mg/m2 once weekly, 4 dose
Maintenance of Remission
- It is important to prevent relapses,
- Azathioprine or methotrexate is used for maintenance therapy
- Azathioprine
- Azathioprine (AZA) is recommended after a 3-month induction period of cyclophosphamide.
- PO, 2 mg/kg for 6-12 months
Methotrexate
- PO,20mg per week for 12 months.
Other
Some medications have never been studied. However, they have been shown to be effective based on findings in case reports. These medications include:
- IV immunoglobulins (Igs)
- Antithymocyte antibody
- Monoclonal antibodies to CD4
- Monoclonal antibodies to CD25
Plasma Exchange
Indications of plasma exchange:
- Removal of circulating auto-antibodies in patients with anti-GBM antibody disease
- Patients with high risk of renal failure
- Patients with serum creatinine > 2.3 mg/dL[3]
- Patients who do not respond to pharmacologic therapy
- Patients diagnosed with pauci-immune crescenteric glomerulonephriits with end stage renal disease and need dialysis[3]
References
- ↑ Nachman PH, Hogan SL, Jennette JC, Falk RJ (1996). "Treatment response and relapse in antineutrophil cytoplasmic autoantibody-associated microscopic polyangiitis and glomerulonephritis". J Am Soc Nephrol. 7 (1): 33–9. PMID 8808107.
- ↑ de Groot K, Harper L, Jayne DR, Flores Suarez LF, Gregorini G, Gross WL, Luqmani R, Pusey CD, Rasmussen N, Sinico RA, Tesar V, Vanhille P, Westman K, Savage CO (May 2009). "Pulse versus daily oral cyclophosphamide for induction of remission in antineutrophil cytoplasmic antibody-associated vasculitis: a randomized trial". Ann. Intern. Med. 150 (10): 670–80. PMID 19451574.
- ↑ 3.0 3.1 Levy JB, Pusey CD (1997). "Still a role for plasma exchange in rapidly progressive glomerulonephritis?". J Nephrol. 10 (1): 7–13. PMID 9241619.