How to Treat Henoch-Schönlein Purpura Nephritis with Crescent Formation

Henoch-Schönlein purpura (HSP) nephritis complicated by crescent formation is classified as rapidly progressive glomerulonephritis and represents one of the most severe manifestations of the disease. This aggressive form demands immediate medical intervention to prevent irreversible kidney damage and improve long-term outcomes.

Understanding Crescentic HSP Nephritis

Crescents in the kidneys develop when inflammatory cells and fibrin accumulate within Bowman's space, leading to structural damage in the glomeruli. When more than 50% of glomeruli show crescentic changes, the condition progresses rapidly, often resulting in acute kidney injury. Early diagnosis through renal biopsy is crucial for initiating timely treatment.

Intensive Immunosuppressive Therapy Approaches

To combat the intense immune-mediated inflammation, a combination of powerful immunosuppressive agents—commonly referred to as triple therapy—is widely recommended. This regimen typically includes:

1. Methylprednisolone Pulse Therapy

High-dose intravenous methylprednisolone at 30 mg/kg per day for three consecutive days helps suppress acute inflammation. This pulse therapy rapidly reduces immune system overactivity and stabilizes glomerular function during the critical early phase.

2. Cyclophosphamide Treatment

Oral or intravenous cyclophosphamide at a dose of 2 mg/kg daily for two months targets abnormal immune cell proliferation. It plays a key role in preventing further autoimmune attacks on the kidneys and is especially effective in severe crescentic cases.

3. Dipyridamole for Antiplatelet and Immunomodulatory Effects

Dipyridamole, administered at 5 mg/kg per day for six months, not only improves microcirculation but also exhibits anti-inflammatory properties that support kidney recovery. Its prolonged use contributes to sustained remission.

Follow-up Oral Steroid Regimen

After the initial intensive phase, patients transition to oral prednisone at 45 mg/m² per day for three months, followed by a gradual tapering schedule. This step-down approach minimizes relapse risk while allowing the body to slowly regain hormonal balance.

Plasma Exchange as an Adjunctive Option

In cases with high levels of circulating immune complexes or antibodies, plasma exchange (plasmapheresis) can be highly beneficial. By filtering harmful proteins and autoantibodies from the bloodstream, this procedure reduces systemic inflammation and provides additional protection to the kidneys.

Managing Acute Renal Failure: The Role of Dialysis

For patients who develop oliguria or anuria leading to acute renal failure, early initiation of dialysis is strongly advised. Peritoneal dialysis is often preferred due to its hemodynamic stability and suitability for pediatric and fragile patients. Prompt dialytic support can bridge the gap until native kidney function begins to recover.

Prognosis and Importance of Timely Intervention

Rapidly progressive HSP nephritis has a poor prognosis if left untreated. The disease evolves quickly, and without aggressive therapy, many patients face life-threatening renal failure within weeks. However, with early detection and comprehensive management—including immunosuppression, plasma exchange, and dialysis when needed—survival rates and renal outcomes significantly improve.

Crescentic HSP nephritis requires a multidisciplinary approach involving nephrologists, immunologists, and critical care specialists. Aggressive treatment protocols must be initiated promptly to halt disease progression and preserve kidney function. Awareness among clinicians and rapid access to diagnostic tools like renal biopsy are essential in turning the tide against this severe form of vasculitis-related kidney disease.