From the Guidelines
The management approach for IGA vasculitis should primarily focus on optimized supportive care, including RAS blockade, blood pressure control, cardiovascular risk minimization, and lifestyle advice, with consideration of glucocorticoid therapy for patients with persistent proteinuria above 0.75-1 g/d despite supportive care. This approach is based on the latest guidelines from the KDIGO 2021 guideline for the management of glomerular diseases 1.
Key Components of Management
- Optimized supportive care is the primary focus, including RAS blockade as much as tolerated or allowed, blood pressure control, cardiovascular risk minimization, adherence to lifestyle advice, including dietary counselling, smoking cessation, weight control, and exercise as appropriate.
- RAS blockade should be instituted irrespective of hypertension if the patient has proteinuria >0.5 g/d.
- For patients with proteinuria that stays above 0.75–1 g/d despite at least 90 days of optimized supportive care, a 6-month course of glucocorticoid therapy may be considered, or preferably, the opportunity to take part in a therapeutic clinical trial.
Considerations for Glucocorticoid Therapy
- Glucocorticoids should be given with extreme caution or avoided entirely in patients with certain conditions, including eGFR <30 ml/min per 1.73 m2, diabetes, obesity, latent infections, secondary disease, active peptic ulceration, uncontrolled psychiatric disease, or severe osteoporosis.
- The use of other immunosuppressive therapies, such as azathioprine, cyclophosphamide, calcineurin inhibitors, and rituximab, is not recommended in IgAN, except in specific situations like rapidly progressive IgAN.
Monitoring and Follow-Up
- Patients require close monitoring of renal function, blood pressure, and urinalysis, with follow-up extending as necessary to detect delayed renal complications.
- Early nephrology consultation is warranted for significant renal involvement as timely intervention can prevent progression to chronic kidney disease.
From the Research
Management Approach for IGA Vasculitis
The management approach for IGA vasculitis involves a combination of symptomatic treatment and immunosuppressive therapy, depending on the severity of the disease.
- For self-limited disease, only symptomatic treatment is recommended 2.
- For severe IgA vasculitis, nephritis, or gastrointestinal manifestations, treatment is not established, but some studies reported a benefit of corticosteroids, combined with immunosuppressive drugs 2, 3, 4.
- The use of corticosteroids and immunosuppressants, such as cyclophosphamide and azathioprine, has been shown to improve renal function and reduce proteinuria in patients with IgA nephropathy associated with crescenteric change 3, 4.
- However, the long-term outcome of IgA vasculitis in adults is difficult to predict, and treatment is often symptomatic because the disease course is usually benign 5.
- Recent studies have shown that corticosteroids have limited effect on long-term outcomes in IgA vasculitis, but steroid-sparing agents have potential for the treatment of recurrent steroid-dependent IgA vasculitis 6.
Treatment Options
- Corticosteroids: high-dose intravenous steroids may be used to treat severe IgA vasculitis, but tapering steroids can lead to recurrence of symptoms 6.
- Immunosuppressants: cyclophosphamide and azathioprine may be used in combination with corticosteroids to treat severe IgA vasculitis and IgA nephropathy 3, 4.
- Symptomatic treatment: may include treatment of gastrointestinal symptoms, arthralgias, and skin manifestations.
Prognosis
- The prognosis of IgA vasculitis depends on the severity of the disease, with severe gastrointestinal and renal involvement being associated with a poorer outcome 2, 5.
- Factors associated with long-term end-stage renal disease include baseline renal function impairment, baseline proteinuria, and degree of interstitial fibrosis, sclerotic glomeruli, and fibrinoid necrosis on renal biopsy 5.