What is the management of different types of glomerulonephritis?

Management of Different Types of Glomerulonephritis

The management of glomerulonephritis should be tailored to the specific type of glomerulonephritis, with treatment regimens designed to address the underlying cause while minimizing immunosuppression-related adverse effects. 1

General Principles of Management

Initial Assessment and Diagnosis

• Kidney biopsy remains the gold standard for diagnosis of glomerular diseases 1
• Evaluate urine sediment for erythrocyte morphology, red cell casts, and acanthocytes 1
• Assess proteinuria with 24-hour urine collection in adults or first morning protein-creatinine ratio in children 1
• Screen for relevant infections: tuberculosis, hepatitis B, hepatitis C, HIV, and syphilis 1

Treatment Selection Framework

1. Choose regimens that avert immediate morbidity based on disease severity and type 1
2. Select treatments that prevent disease progression - complete remission may not always be possible 1
3. Minimize harmful side effects from immunosuppression 1

Management of Specific Types of Glomerulonephritis

1. Post-Streptococcal Glomerulonephritis (PSGN)

• Primary treatment is supportive care as the disease typically follows a self-limiting course 2
• Antibiotic therapy (penicillin or erythromycin if penicillin-allergic) for 10 days to eliminate remaining streptococcal organisms 2
• Management of hypertension and edema:
  • Sodium restriction (<2.0 g/day) 1, 2
  • Diuretics (loop or thiazide) as first-line treatment 2
  • Monitor for adverse effects: hyponatremia, hypokalemia, GFR reduction 2
• Avoid immunosuppressive therapy in typical PSGN 2

2. Hepatitis-Related Glomerulonephritis

Hepatitis B-Related GN

• Treatment with nucleoside analogues (tenofovir or entecavir) is recommended as first-line therapy 1
• Interferon-alfa is an alternative but less commonly used due to safety profile and administration challenges 1
• Note that nucleoside analogue therapy would typically be required for life, while interferon may potentially lead to cure 1

Hepatitis C-Related GN

• For CKD Stages 1-2: Combined antiviral treatment with pegylated interferon and ribavirin 1
• For CKD Stages 3-5 (not on dialysis): Monotherapy with pegylated interferon, with doses adjusted to kidney function 1
• Newer protease inhibitors (boceprevir or telaprevir) may increase response rates from 40-50% to 70-80% 1

3. Membranoproliferative Glomerulonephritis (MPGN)

• Modern classification is based on complement mechanisms rather than histology:
  • Immunoglobulin-positive (Ig+C3+)
  • Immunoglobulin-negative (Ig-C3+) including dense deposit disease and C3 nephropathy 1
• For idiopathic MPGN with nephrotic syndrome AND progressive decline in kidney function:
  • Oral cyclophosphamide or mycophenolate mofetil (MMF) plus low-dose corticosteroids 1
  • Limit initial therapy to less than 6 months 1
• Treatment should target the underlying cause when identified 1

4. Rapidly Progressive Glomerulonephritis (RPGN)

• Standard management includes corticosteroids and cytotoxic drugs 3
• Rituximab may be considered for:
  • Disease refractory to standard management
  • Patients with intolerable side effects to standard therapy 3
• Rituximab is not recommended as first-line therapy based on current evidence 3

Supportive Care for All Types of Glomerulonephritis

Blood Pressure Management

• ACE inhibitors or ARBs at maximally tolerated doses for patients with hypertension and proteinuria 1
• Target systolic BP <120 mmHg in most adult patients 1
• Target 24-hour mean arterial pressure ≤50th percentile for age, sex, and height in children 1

Edema Management

• Dietary sodium restriction (<2.0 g/day) 1, 2
• Diuretics as first-line therapy 1
• If response is insufficient, add mechanistically different diuretics 1
• Monitor for adverse effects: hyponatremia, hypokalemia, GFR reduction, volume depletion 1

Dietary Management

• Protein restriction based on proteinuria level and kidney function:
  • Nephrotic-range proteinuria: 0.8-1 g/kg/day 1
  • eGFR <60 ml/min/1.73 m² with nephrotic-range proteinuria: limit to 0.8 g/kg/day 1
  • Avoid <0.6 g/kg/day due to malnutrition risk 1
• Plant-based protein sources are preferred 1
• Target caloric intake: 35 kcal/kg/day (30-35 kcal/kg/day if eGFR <60) 1

Infection Prevention

• Pneumococcal vaccine for patients with glomerular disease, nephrotic syndrome, or CKD 1
• Influenza vaccine for patients and household contacts 1
• Herpes zoster vaccination (Shingrix) 1
• Consider prophylactic trimethoprim-sulfamethoxazole for patients on high-dose prednisone, rituximab, or cyclophosphamide 1
• Screen for Strongyloides in patients from endemic areas with eosinophilia and elevated IgE before immunosuppression 1

Monitoring and Follow-up

• Monitor proteinuria reduction as a surrogate endpoint in treatment 1
• A ≥40% decline in eGFR from baseline over 2-3 years suggests progression to kidney failure 1
• Monitor for development of cancers or infections with prolonged immunosuppression 1

Common Pitfalls to Avoid

• Failure to screen for infections before immunosuppression
• Inadequate dose adjustment of medications based on kidney function
• Overlooking fertility preservation options before cytotoxic therapy
• Not providing prophylaxis against specific immunosuppressive drug side effects
• Neglecting vaccination status before immunosuppression
• Using outdated classification systems (e.g., MPGN) rather than mechanism-based approaches