How to Manage Henoch-Schönlein Purpura Nephritis: Symptoms, Treatment, and Long-Term Outlook
Henoch-Schönlein purpura nephritis (HSPN) is a kidney complication that arises from Henoch-Schönlein purpura (HSP), an autoimmune condition characterized by inflammation of small blood vessels. This disorder primarily affects children but can also occur in adults. The hallmark of HSPN is the deposition of immunoglobulin A (IgA) immune complexes in the glomeruli—the filtering units of the kidneys—leading to visible signs such as hematuria (blood in urine) and proteinuria (excess protein in urine).
Understanding the Pathophysiology of HSPN
The underlying mechanism involves abnormal immune responses where IgA-containing immune complexes accumulate in the kidney tissue. This triggers inflammation and damage to the glomerular basement membrane, impairing normal kidney function. Over time, persistent inflammation may lead to chronic kidney disease if not properly managed.
Key Clinical Goals in HSPN Management
Effective treatment focuses on two primary targets: maintaining blood pressure below 130/80 mmHg and reducing the 24-hour urinary protein excretion to less than 0.5 grams. Achieving these benchmarks significantly improves long-term renal outcomes and reduces the risk of progressive kidney damage.
First-Line Therapy: ACE Inhibitors and ARBs
Angiotensin-converting enzyme inhibitors (ACEIs) or angiotensin II receptor blockers (ARBs) are considered first-line medications due to their dual benefits. These drugs not only help regulate blood pressure but also reduce intraglomerular pressure, thereby decreasing protein leakage into the urine. Commonly prescribed options include lisinopril, enalapril, losartan, and valsartan. Patients typically require consistent use over several months to assess full therapeutic response.
When First-Line Treatment Isn't Enough
If after three months of optimized ACEI or ARB therapy the patient fails to meet target levels for blood pressure and proteinuria, additional interventions become necessary. In such cases, corticosteroids like prednisone may be introduced. Steroid therapy helps suppress the overactive immune response contributing to kidney inflammation and has been shown to improve renal parameters in refractory cases.
Supportive Care and Monitoring
Beyond pharmacological treatment, regular monitoring through urinalysis, serum creatinine tests, and blood pressure checks is essential. Lifestyle modifications—including a low-sodium diet, adequate hydration, and avoidance of nephrotoxic substances—can further support kidney health. Pediatric patients often have a better prognosis, with many experiencing spontaneous remission, while adults may face a higher risk of persistent disease.
Prognosis and Future Considerations
Most individuals with mild HSPN recover fully without lasting effects. However, those with severe histological findings on kidney biopsy—such as crescentic glomerulonephritis—may require more aggressive immunosuppressive regimens involving agents like cyclophosphamide or mycophenolate mofetil. Ongoing research continues to explore targeted biologic therapies that modulate IgA production and immune complex clearance.
Early diagnosis, individualized treatment plans, and close follow-up are critical in minimizing complications and preserving kidney function in patients with Henoch-Schönlein purpura nephritis.