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Immunosuppressive treatment in immunoglobulin a nephropathy
When should immunosuppressive therapy be considered?
Persistent proteinuria
In patients with persistent proteinuria (>1000 mg/day) despite optimal conservative therapy (ACEI or ARB) with preserved kidney function, earlier studies have shown benefit of a 6-month course of steroid therapy in patients, with a lower risk of side effects.
However, a randomized controlled trial (STOP-IgA) has cast doubt on the short-term benefit of preserving GFR, even when steroid treatment showed significant improvement in proteinuria.
Steroid side effects associated with such courses include glucose intolerance and a higher risk of infection.
Nephrotic syndrome
In patients presenting with nephrotic syndrome and clinical/pathologic features suggestive of concurrent MCD, corticosteroids prescribed in a manner similar to that used in the treatment of MCD alone will commonly induce a complete remission of proteinuria.
Accelerated decline in GFR
In patients with a rapidly declining GFR (to less than 50 mL/min per 1.73 m 2 ), a biopsy can be helpful in risk stratification.
If advanced sclerosis and fibrosis is found, the risk of immunosuppression usually outweighs the benefit. However, if significant inflammatory activity is seen with less sclerosis and fibrosis, immunosuppression can halt or substantially reduce the rate of decline of kidney function.
The evidence to guide treatment in this group of patients is scarce and limited. With higher grades of activity, additional treatment beyond a course of prednisone—such as cyclophosphamide (typically for 3 months)—has been used, especially when glomerular crescents are found.
Further maintenance treatment and/or steroid-sparing agents in the form of azathioprine or mycophenolate mofetil have been used, but no randomized controlled trials have confirmed their value.
Resistant and relapsed disease
In some patients, first-line immunosuppression (steroid monotherapy or in combination with cyclophosphamide) may fail to diminish proteinuria. In other patients, relapse after the achievement of remission may occur shortly after an immunosuppressive course is completed or after many years.
In such situations, additional courses of immunotherapy increase their risk, and a kidney biopsy may be needed to define the activity versus chronicity of the disease and help guide treatment.
Advanced disease
Advanced glomerular sclerosis and interstitial fibrosis usually follow chronic unremitting IgAN or acute/subacute aggressive variants of the disease. Such irreversible findings on biopsy will be reflected in the declining GFR.
The level of sclerosis/fibrosis and GFR at which the risk of therapy outweighs the benefit is unclear and remains a subject of debate.
The Kidney Disease: Improving Global Outcomes (KDIGO) guidelines suggest to withhold immunosuppressive treatment if the GFR is less than 30 mL/min per 1.73 m 2 unless active crescentric IgAN is found.
Although these recommendations are not based on strong evidence, they are reflective of general practice.
