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
Treatment for rapidly progressive glomerulonephritis 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. Medical therapy focuses on to induce and then maintain the remission. Two pharmacologic agents used for induction of remission in patients with rapidly progressive glomerulonephritis are glucocorticoids (high dose) and cyclophosphamide. Cyclophosphamide is necessary to prevent relapse. Azathioprine or methotrexate is used for maintenance therapy. Azathioprine (AZA) is recommended after a 3-month induction period of cyclophosphamide.Indications of plasma exchange include, anti-GBM antibody disease, antineutrophil cytoplasmic antibody (ANCA)-associated rapidly progressive glomerulonephritis, patients with high risk of renal failure, patients with serum creatinine > 2.3 mg/dL, those patients who do not respond to pharmacologic therapy, and patients diagnosed with pauci-immune crescenteric glomerulonephriits with end stage renal disease and need dialysis. Risk and complications of plasma exchange are transient fall in blood-clotting factors, mild prolongation of prothrombin and activated partial thromboplastin times. Clinically significant bleeding is rare but a coagulation screen should be undertaken before surgery or organ biopsy is performed. Other risks include haematomas at venepuncture/line insertion sites, vasovagal episodes with fainting, fluid overload or under-replacement, and allergic or anaphylactic reactions due to plasma infusion.
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Medical therapy
Medical therapy focuses on 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;
- Glucocorticoids (high dose)
- Cyclophosphamide
Glucocorticoids[1]
- Preffered regimen (1) : Methylprednisolone, 7 mg/kg/d, Intravenous at a maximum dose of 1 g for 3 days
- preffered regimen (2) :Prednisone, 1 mg/kg/d ,PO at a maximum dose of 80 mg for 3 weeks.
Then 2 mg/kg/d, PO at a maximum dose of 120 mg for 3 months.
Then taper patient off steroids by deceasing 25% of prednisone dose every 4 weeks until patient stops
Immunosuppressive Therapy
- Preffered regimen(1) : Cyclophosphamide: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[2]
- Preffered regimen(2) :Cyclophosphamide PO:1.5- 2 mg/kg/day until remission is achieved, followed by 1.5 mg/kg/day for 3 more months.
- Preffered regimen(3) Rituximab IV, 375 mg/m2 once weekly, 4 dose
Maintenance of Remission
- Preffered regimen (1) , Azathioprine, PO, 2 mg/kg for 6-12 months
- Preffered regimen (2) :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[3]:
- Anti-GBM antibody disease
- Antineutrophil cytoplasmic antibody (ANCA)-associated rapidly progressive glomerulonephritis
- Patients with high risk of renal failure
- Patients with serum creatinine > 2.3 mg/dL[4]
- Patients who do not respond to pharmacologic therapy
- Patients diagnosed with pauci-immune crescenteric glomerulonephriits with end stage renal disease and need dialysis[4]
Risk and complications of plasma exchange
- Transient fall in blood-clotting factors
- Mild prolongation of prothrombin time and activated partial thromboplastin times.
- Haematomas at venepuncture/line insertion sites
- Vasovagal episodes with fainting
- Fluid overload or under-replacement
- Allergic or anaphylactic reactions due to plasma infusion.
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.
- ↑ Apaydin S (February 2018). "The treatment of ANCA-associated rapidly-progressive glomerulonephritis and Goodpasture syndrome with therapeutic apheresis". Transfus. Apher. Sci. 57 (1): 8–12. doi:10.1016/j.transci.2018.02.007. PMID 29503131.
- ↑ 4.0 4.1 Levy JB, Pusey CD (1997). "Still a role for plasma exchange in rapidly progressive glomerulonephritis?". J Nephrol. 10 (1): 7–13. PMID 9241619.