From the Guidelines
Henoch-Schönlein Purpura (HSP) typically follows a self-limiting course with complete resolution in most patients, with the majority of children experiencing complete resolution within 2 months without specific treatment beyond supportive care. The disease usually begins with an initial phase characterized by the classic tetrad of palpable purpura, arthralgia/arthritis, abdominal pain, and renal involvement. The acute phase generally lasts 4-6 weeks, during which symptoms may fluctuate in intensity.
Key Characteristics of HSP
- Most children (approximately 80%) experience complete resolution within 2 months without specific treatment beyond supportive care 1
- About 20-40% of patients experience recurrences, typically within 6 weeks of the initial episode, though recurrences can occur up to 2 years later 1
- These recurrences are usually milder and shorter than the initial presentation
- The long-term prognosis is generally excellent, with mortality being extremely rare
- The most significant long-term complication is renal disease, which affects about 1-5% of patients who may develop chronic kidney disease or end-stage renal disease
Risk Factors for Poor Renal Outcomes
- Persistent proteinuria
- Nephrotic syndrome
- Decreased GFR at presentation
- Crescentic glomerulonephritis on biopsy
Monitoring and Treatment
- Regular follow-up with urinalysis for at least 6 months after diagnosis is recommended to monitor for renal involvement, with more prolonged monitoring for those who develop nephritis 1
- There is moderate quality evidence that corticosteroids are not beneficial at the onset of HSP to prevent nephritis or decrease the risk of severe persistent nephritis or relapsing disease 1
- Immunosuppressant therapy may be considered for patients with severe renal involvement, but the choice of medication should be individualized and based on the patient's specific needs and circumstances 1
From the Research
Natural History of Henoch-Schönlein Purpura (HSP)
- HSP is an acute, systemic, immune complex-mediated, leukocytoclastic vasculitis characterized by a triad of palpable purpura, abdominal pain, and arthritis 2.
- The condition is more common in children younger than 10 years, but adults are more likely to experience complications 2.
- The clinical manifestations of HSP include:
- Purpuric rash (100% of patients)
- Arthritis (75% of patients)
- Abdominal pain (60-65% of patients)
- Renal disease (40-50% of patients) 2
- The natural history of HSP is generally self-limiting, with spontaneous resolution in 94% of children and 89% of adults 2.
Prognosis and Long-term Complications
- The long-term prognosis of HSP depends on the severity of renal involvement 2, 3.
- End-stage renal disease occurs in 1-5% of patients 2.
- Proteinuria adversely affects the outcome, and early aggressive therapy with high-dose steroids plus immunosuppressants is recommended for patients with severe renal involvement 3.
- Long-term complications are rare and include persistent hypertension and end-stage kidney disease 4.
Treatment and Management
- Supportive treatment is the primary intervention for HSP, with oral prednisone used to treat abdominal and joint symptoms 2.
- Corticosteroids may be considered for HSP patients with severe gastrointestinal pain and gastrointestinal hemorrhage 4.
- The use of corticosteroids for prevention and treatment of renal disease in children with HSP is still debated, with some evidence suggesting they are not effective in preventing renal involvement but may be useful in treating renal involvement 5, 6.
- Angiotensin-converting enzyme inhibitors or angiotensin receptor blockers may be considered in addition to corticosteroids to prevent and/or limit secondary glomerular injury in children with severe nephritis or renal involvement 4.