Initial Treatment Approach for Nephrotic and Nephritic Syndrome

Diagnostic Approach

The American Academy of Pediatrics and Kidney International recommend confirming nephrotic-range proteinuria with 24-hour urine collection or spot urine protein-to-creatinine ratio 2
Investigations should be performed to exclude secondary causes, such as diabetes, lupus, infections, and medications, as suggested by Kidney International 2
In adults, kidney biopsy is generally indicated before initiating immunosuppressive therapy, according to Kidney International 2
In children with typical presentation, biopsy may be deferred if there is response to initial steroid therapy, as recommended by the American Academy of Pediatrics and Kidney International 2, 3
Biopsy is indicated in steroid-resistant nephrotic syndrome in children, as suggested by Kidney International 4

The Kidney International guidelines recommend administering prednisone/prednisolone at 1 mg/kg/day (maximum 80 mg) or alternate-day dose of 2 mg/kg (maximum 120 mg) for adults 1, 2
High-dose corticosteroids should be continued for a minimum of 4 weeks if complete remission is achieved, and up to 16 weeks if remission is not achieved, as recommended by Kidney International 1
After achieving remission, steroids should be tapered slowly over a period of up to 6 months, according to Kidney International 1

Steroid resistance is defined after a minimum of 8 weeks of treatment with corticosteroids, as suggested by Kidney International 4
For steroid-resistant cases, calcineurin inhibitors (CNIs) are recommended as initial therapy, according to Kidney International 4
CNI therapy should be continued for a minimum of 6 months and then stopped if remission is not achieved, as recommended by Kidney International 4
If at least partial remission is achieved by 6 months, continue CNIs for a minimum of 12 months, according to Kidney International 4

Pneumococcal vaccination should be administered, as recommended by the American Academy of Pediatrics and Kidney International 2
Annual influenza vaccination should be given to patients and household contacts, according to the American Academy of Pediatrics and Kidney International 2
Live vaccines are contraindicated in children receiving immunosuppressive agents, as suggested by Kidney International 4

Regularly assess proteinuria and kidney function to evaluate treatment response, as recommended by the American Academy of Pediatrics and Kidney International 2
Monitor for side effects of medications, particularly with long-term immunosuppressive therapy, according to the American Academy of Pediatrics and Kidney International 2