Effective Treatment Strategies for Henoch-Schönlein Purpura Nephritis (HSPN)

Henoch-Schönlein purpura nephritis (HSPN) is a serious kidney complication arising from IgA-mediated small-vessel vasculitis—commonly triggered by infections, medications, or environmental allergens. Left untreated, it can progress to chronic kidney disease or even end-stage renal failure. Fortunately, with early diagnosis and a personalized, multi-tiered therapeutic approach, most patients achieve remission and preserve long-term renal function.

Comprehensive, Evidence-Based Treatment Framework

1. Etiology-Directed Intervention: Eliminate the Trigger

Identifying and removing the underlying cause is the critical first step. Bacterial or viral infections—especially upper respiratory tract infections—are the most common precipitants. In such cases, targeted antimicrobial therapy (e.g., penicillin or macrolides for streptococcal infection) is initiated. For suspected drug-induced HSPN, immediate discontinuation of the offending agent—such as NSAIDs, antibiotics, or herbal supplements—is essential. Environmental triggers like seasonal pollen or food additives may also warrant detailed allergy testing and avoidance strategies.

2. Allergen Management & Anti-Inflammatory Support

While true IgE-mediated food allergy is rare in HSPN, dietary sensitivities can exacerbate inflammation. A short-term elimination diet—guided by an allergist or registered dietitian—may help identify potential contributors (e.g., dairy, eggs, nuts, or gluten). Concurrently, second-generation antihistamines (like loratadine or cetirizine) provide symptomatic relief for urticarial rashes and pruritus without sedation. In moderate-to-severe cutaneous involvement, short-course systemic corticosteroids (e.g., prednisone 0.5–1 mg/kg/day for 7–14 days) significantly reduce rash duration and gastrointestinal symptoms.

3. Symptom-Specific Supportive Care

Abdominal pain, arthralgia, and gastrointestinal bleeding are hallmark extrarenal manifestations. Non-opioid analgesics like acetaminophen are preferred; NSAIDs are avoided due to their nephrotoxic potential and risk of worsening glomerular injury. For severe colicky pain or GI vasculitis, low-dose oral corticosteroids offer rapid anti-inflammatory control. Joint swelling typically resolves with rest and supportive care—though persistent synovitis may require rheumatology consultation to rule out overlapping autoimmune conditions.

4. Immunosuppressive Therapy: Tailored to Renal Risk Stratification

Renal involvement severity—assessed via urine protein-to-creatinine ratio (UPCR), estimated GFR, and especially kidney biopsy findings—dictates immunosuppression intensity. Patients with isolated microscopic hematuria or mild proteinuria (<0.5 g/day) often respond well to ACE inhibitors (e.g., lisinopril) or ARBs (e.g., losartan) to reduce intraglomerular pressure and proteinuria.

For those with nephrotic-range proteinuria (>3.5 g/day), rapidly rising serum creatinine, or histopathologic evidence of crescentic glomerulonephritis, aggressive immunomodulation is indicated. First-line regimens include:

• Oral corticosteroids (e.g., prednisone 1–1.5 mg/kg/day tapered over 3–6 months);
• Combination therapy (steroids + mycophenolate mofetil or azathioprine) for steroid-dependent or relapsing cases;
• Cyclophosphamide pulses in life-threatening crescentic GN or rapidly progressive renal failure.

5. Advanced Interventions for Critical Cases

In patients presenting with acute kidney injury (AKI), dialysis dependence, or >50% cellular crescents on biopsy, plasma exchange (PLEX) is recommended alongside high-dose IV methylprednisolone and cyclophosphamide. Emerging biologics—including rituximab (anti-CD20) and targeted complement inhibitors—are under active investigation in clinical trials for refractory HSPN and show promising early results in reducing B-cell-driven IgA deposition.

Long-term monitoring—including serial UPCR, serum creatinine, blood pressure, and eGFR—is vital for detecting late relapses or silent progression. With timely, stratified care, over 90% of children and 75–85% of adults achieve complete or partial remission within 12 months. Partnering with a nephrologist experienced in vasculitis management significantly improves outcomes and quality of life.