From the Guidelines
HSP, or Henoch-Schönlein Purpura, is an autoimmune disorder characterized by inflammation of small blood vessels, primarily affecting children between ages 2-10, and is treated with supportive care, including rest, adequate fluid intake, and pain management with acetaminophen or ibuprofen for mild cases.
Key Characteristics of HSP
- It causes a distinctive purplish rash (purpura) on the lower extremities and buttocks, along with joint pain, abdominal pain, and sometimes kidney involvement.
- The condition occurs when the immune system abnormally deposits IgA antibodies in blood vessel walls, triggering inflammation.
- Regular monitoring of blood pressure and urine tests is important during the acute phase to detect potential kidney complications early, as suggested by 1.
Treatment Approach
- For severe symptoms, especially with significant kidney involvement, corticosteroids like prednisone may be prescribed, but the long-term benefit of steroid therapy for nephrotic-range or persistent proteinuria has not been proved, as noted in 1.
- The use of ACE inhibitors or ARBs in children with HSP and persistent significant proteinuria is considered, based on extrapolation of results with IgAN, as discussed in 1.
Management Considerations
- Renal biopsy should be performed in children with decreased renal function at presentation or with severe NS/nephritic syndrome, as recommended in 1.
- Therapeutic interventions should be confined to those children with NS or nephritic syndrome rather than isolated proteinuria exceeding 1 g/d, or those with persistent heavy proteinuria during follow-up, as suggested by 1.
- The threshold for corticosteroid treatment in children with HSP should not be solely based on proteinuria levels, and additional studies are needed before a recommendation can be made to administer corticosteroids to patients with HSP nephritis who have lower levels of proteinuria, as stated in 1.
From the Research
Definition of HSP
- Henoch-Schönlein purpura (HSP) is an acute, systemic, immune complex-mediated, leukocytoclastic vasculitis 2
- It is characterized by a triad of palpable purpura, abdominal pain, and arthritis 2
- HSP is also known as immunoglobulin A (IgA) vasculitis, and is a systemic, immune complex-mediated, small-vessel leukocytoclastic vasculitis 3
Clinical Manifestations
- The clinical manifestations of HSP are thought to arise from IgA depositions in blood vessel walls in the affected organs, mostly skin, gastrointestinal tract, joints, and kidneys 4
- HSP occurs most often in children between the ages of 3 and 10 years, and presents classically with a unique distribution of the rash to the lower extremities and the buttocks area 5
- All patients with HSP develop a purpuric rash, 75% develop arthritis, 60 to 65% develop abdominal pain, and 40 to 50% develop renal disease 2
Treatment and Prognosis
- HSP spontaneously resolves in 94% of children and 89% of adults, making supportive treatment the primary management strategy 3
- Corticosteroids may be effective in rapid resolution of renal manifestations and treating joint and abdominal pain, but they are not proven effective for treating organ manifestations and complications 4
- Mycophenolate mofetil or cyclosporine A may be better treatment choices in case of renal involvement 4
- Long-term prognosis depends on the extent of renal involvement, and end-stage renal disease occurs in 1 to 5% of patients 2