From the Guidelines
HSP, or Henoch-Schönlein Purpura, is a condition characterized by inflammation of small blood vessels, leading to leakage of blood, and is also known as IgA vasculitis. This condition primarily affects children and is marked by a distinctive purple rash on the legs and buttocks, accompanied by joint pain, abdominal pain, and sometimes kidney problems 1. The immune system's abnormal deposition of IgA antibodies in blood vessel walls triggers the inflammation 1.
Key Characteristics and Treatment
- The condition typically resolves on its own within 4-6 weeks with supportive care, including rest, adequate fluid intake, and pain management with acetaminophen or ibuprofen.
- More severe cases, especially those with significant kidney involvement, may require corticosteroids like prednisone, typically at a dose of 1-2 mg/kg/day for 1-2 weeks, then tapered 1.
- Children with HSP nephritis and persistent proteinuria may be treated with ACEi or ARBs, as suggested by guidelines 1.
- About one-third of patients may experience recurrences, and those with significant kidney involvement should have follow-up urinalysis and blood pressure monitoring for 6-12 months to detect any long-term complications.
Clinical Considerations
The approach to treatment in adults does not differ significantly from that in children, although the long-term benefit of steroid therapy for nephrotic-range or persistent proteinuria, or intravenous corticosteroids and other immunosuppressive agents for crescentic HSP in adults, has not been proven 1. The prognosis in adult HSP is worse with protein excretion of 1 g/d, indicating the importance of monitoring and managing kidney involvement 1.
From the Research
Definition of HSP
- HSP stands for Henoch-Schönlein Purpura, also known as immunoglobulin A (IgA) vasculitis 2, 3, 4, 5, 6.
- It is a systemic, immune complex-mediated, small-vessel leukocytoclastic vasculitis characterized by nonthrombocytopenic palpable purpura, arthritis, and abdominal pain 2.
- IgA vasculitis is caused by the deposition of immune complexes in small blood vessels, including the renal glomeruli and mesangium 4.
Clinical Manifestations
- The clinical association of purpura, arthralgia, and arthritis was first described in 1837 by Johann Lukas Schönlein 4.
- HSP is characterized by a unique distribution of the rash to the lower extremities and the buttocks area, especially in children 3.
- Clinical manifestations mainly involve cutaneous purpura, arthralgias and/or arthritis, acute enteritis, and glomerulonephritis 5, 6.
Diagnosis and Treatment
- Diagnostic testing is required only to exclude other etiologies of purpura, to identify renal involvement, and, if indicated, to determine its extent with biopsy 2.
- Detection of IgA deposition in small blood vessels and the renal glomeruli is diagnostic in most cases 4.
- Treatment is often symptomatic because the disease course is usually benign, but severe involvement may require immunosuppressive therapy 5, 6.
- The use of steroids, cyclosporine, and mycophenolate has been demonstrated to be successful in treating glomerulonephritis and other complications 2.