From the Guidelines
Henoch-Schönlein purpura nephritis (HSPN) patients with persistent proteinuria should be treated with ACEi or ARBs, as suggested by the 2012 KDIGO practice guideline on glomerulonephritis 1.
Treatment Overview
Treatment of HSPN depends on the severity of the disease, with mild cases often requiring only supportive care and monitoring.
- For patients with significant proteinuria or declining kidney function, the following treatments may be considered:
- Corticosteroids like prednisone at 1-2 mg/kg/day for 4-8 weeks followed by a gradual taper
- In severe cases with rapidly progressive glomerulonephritis or nephrotic syndrome, more aggressive therapy may be needed, including high-dose pulse methylprednisolone (30 mg/kg/day for 3 days) followed by oral prednisone
- Possibly immunosuppressants like cyclophosphamide, azathioprine, or mycophenolate mofetil
- ACE inhibitors or ARBs are often added to reduce proteinuria and protect kidney function, as supported by the commentary on the 2012 KDIGO clinical practice guideline for glomerulonephritis 1
Disease Pathophysiology and Monitoring
The condition results from IgA immune complex deposition in the kidneys, triggering inflammation and potential kidney damage, which explains why immunosuppressive therapy is the mainstay of treatment for moderate to severe cases.
- Regular monitoring of blood pressure, urinalysis, and kidney function is essential, as approximately 20% of children with HSPN may develop chronic kidney disease.
- Renal biopsy should be performed in children with decreased renal function at presentation or with severe NS/nephritic syndrome, as recommended in the commentary on the 2012 KDIGO clinical practice guideline for glomerulonephritis 1
Immunossuppressant Therapy
The use of immunosuppressant therapy for HSPN is center-specific and a unanimous practice algorithm does not exist, with a preference to use immunosuppressant therapy that combines steroid therapy with one of azathioprine, cyclosporine, tacrolimus, or MMF/mycophenolic acid, as stated in the Canadian Society of Nephrology commentary on the 2012 KDIGO clinical practice guideline for glomerulonephritis 1
- Cyclophosphamide is used less frequently, particularly because there is no evidence suggesting increased efficacy and due to side effects of infertility and malignancy.
- Cost has a significant impact on the choice of immunosuppressant therapy.
From the Research
Henoch-Schönlein Nephritis
- Henoch-Schönlein nephritis is a rare kidney disease that can lead to chronic kidney disease in a significant percentage of patients 2.
- The prognosis of Henoch-Schönlein purpura (HSP) nephritis is more severe than originally thought, with a significant portion progressing to deterioration of renal function in adulthood 3.
- Proteinuria adversely affects the outcome of HSP nephritis, and patients with nephrotic levels of proteinuria at onset are more likely to progress to end-stage renal disease (ESRD) 4.
Treatment Options
- Treatment of HSP nephritis can range from simply steroids to combined immunosuppressant treatments 5.
- Methylprednisolone pulse therapy and prednisone per os are tested drugs, and can be used in combination with other immunosuppressant drugs, such as cyclosporin A and cyclophosphamide 5.
- Cyclophosphamide has been demonstrated to be effective in a recent randomized controlled trial 5.
- Angiotensin-converting enzyme (ACE) inhibitors can be used to treat HSP nephritis, regardless of blood pressure values 3, 6.
Disease Course and Outcome
- The disease course and outcome of HSP nephritis can vary widely, with some patients experiencing spontaneous recovery and others progressing to ESRD 4, 2.
- Nephrotic syndrome, decreased glomerular filtration rate (GFR), and more severe histopathology at onset are ominous signs for the outcome of HSP nephritis 4.
- Late initiation of treatment is associated with an increased risk for persistent proteinuria 6.
- Long-term follow-up of HSP nephritis patients is mandatory, as urinary abnormalities may persist or develop over time 6, 2.