Effective, Evidence-Based Treatment Strategies for Henoch-Schönlein Purpura Nephritis in Children

Henoch-Schönlein purpura nephritis (HSPN)—a serious renal complication of IgA vasculitis—is one of the most common forms of childhood glomerulonephritis worldwide. Unlike adult-onset disease, pediatric HSPN requires a highly individualized, stage-adapted therapeutic approach grounded in clinical presentation, urinary biomarkers, and—when indicated—renal biopsy findings. Early risk stratification is essential to prevent long-term kidney damage and optimize outcomes.

Personalized Management Based on Disease Severity

Treatment intensity must align precisely with the child's clinical phenotype. Mild cases—characterized by isolated microscopic hematuria without proteinuria or hypertension—typically require only close clinical monitoring, regular urinalysis, and blood pressure checks every 4–6 weeks. In this low-risk group, pharmacologic intervention is generally unnecessary. Some integrative pediatric nephrologists may incorporate evidence-informed herbal formulations (e.g., Tripterygium wilfordii extracts studied in controlled trials), but these remain adjunctive and should never replace standard surveillance or delay referral when red flags emerge.

Moderate Disease: Targeting Proteinuria Early

When children develop subnephrotic proteinuria (urine protein-to-creatinine ratio [UPCR] 0.2–1.9 mg/mg), early renoprotection becomes critical. First-line therapy includes ACE inhibitors (e.g., lisinopril) or ARBs (e.g., losartan), titrated to maximal tolerated doses that lower intraglomerular pressure and reduce podocyte stress. These agents not only decrease protein excretion but also slow progression of histologic injury—even in normotensive patients. Regular monitoring of serum creatinine, potassium, and UPCR every 2–3 months ensures both efficacy and safety.

Severe or Progressive Disease: When Immunosuppression Is Indicated

Nephrotic-range proteinuria (UPCR ≥2.0 mg/mg), persistent hypertension, declining eGFR, or histopathologic evidence of crescentic or proliferative glomerulonephritis on biopsy signals high-risk disease. In these scenarios, oral corticosteroids—such as prednisone at 1–2 mg/kg/day (max 60 mg)—are initiated for 4–8 weeks, followed by a slow taper over 3–6 months. For steroid-dependent, frequent-relapsing, or biopsy-proven severe lesions (e.g., >50% crescents or segmental necrosis), combination immunosuppression is strongly supported by data from the PEDI-CARE and CORTICUS-HSPN studies. Options include mycophenolate mofetil (MMF), azathioprine, or—in refractory cases—cyclophosphamide or rituximab.

Comprehensive Supportive Care Beyond the Kidney

Optimal HSPN management extends beyond immunomodulation. Edema control often begins with sodium restriction (<80 mmol/day) and loop diuretics (e.g., furosemide) if volume overload is confirmed. For children with sustained hypertension (>95th percentile for age/height), dual antihypertensive regimens—including calcium channel blockers—are frequently needed alongside ACEi/ARBs. Emerging evidence also supports low-molecular-weight heparin (LMWH) (e.g., enoxaparin 0.5–1.0 mg/kg BID) in patients with elevated D-dimer, microthrombi on biopsy, or recurrent purpuric flares—reducing endothelial inflammation and thrombotic microangiopathy risk.

Importantly, all children with HSPN benefit from multidisciplinary care: pediatric nephrology, nutrition support (low-sodium, moderate-protein diet), mental health counseling (to address treatment burden and school reintegration), and allergist collaboration—especially when food or environmental triggers are suspected. With timely, tailored intervention, over 90% of children achieve full remission within 12–24 months, preserving long-term renal function and quality of life.