From the Guidelines
Henoch-Schönlein purpura (HSP) treatment should focus on supportive care, with corticosteroids reserved for severe symptoms, as there is no evidence to suggest that the approach to treatment in adults should differ from that in children 1.
Overview of HSP
HSP, now known as IgA vasculitis, is a small vessel vasculitis that primarily affects children but can occur in adults. It is characterized by palpable purpura (non-blanching rash) typically on the lower extremities and buttocks, arthralgia or arthritis, abdominal pain, and kidney involvement.
Treatment Approach
Treatment is largely supportive for most cases, including:
- Adequate hydration
- Rest
- Pain management with acetaminophen or NSAIDs (unless contraindicated by kidney involvement or GI symptoms) For severe symptoms, corticosteroids may be used: prednisone 1-2 mg/kg/day for 1-2 weeks with gradual tapering.
Severe Kidney Involvement
Severe kidney involvement might require higher doses of steroids, immunosuppressants like cyclophosphamide or azathioprine, or even plasmapheresis in resistant cases.
Prognosis and Monitoring
Most children recover completely within 4-6 weeks, though recurrences can occur in about one-third of patients. The condition results from IgA immune complex deposition in small blood vessels, often triggered by upper respiratory infections, certain medications, or foods. Regular monitoring of blood pressure and urinalysis is important to detect kidney complications, which can lead to long-term renal problems in a small percentage of patients.
Corticosteroids Use
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.
From the Research
Definition and Characteristics
- 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 IgA vasculitis, a systemic, immune complex-mediated, small-vessel leukocytoclastic vasculitis 3
Clinical Manifestations
- The disease is characterized by nonthrombocytopenic palpable purpura, arthritis, and abdominal pain 3
- All patients with HSP develop a purpuric rash, 75% develop arthritis, 60-65% develop abdominal pain, and 40-50% develop renal disease 2
- The clinical manifestations are thought to arise from IgA depositions in blood vessel walls in the affected organs, mostly skin, gastrointestinal tract, joints, and kidneys 4
Diagnosis
- The diagnosis should be based on the finding of palpable purpura in the presence of at least one of the following criteria: diffuse abdominal pain, arthritis or arthralgia, renal involvement, and a biopsy showing predominant IgA deposition 5
- Diagnostic testing is required only to exclude other etiologies of purpura, to identify renal involvement, and, if indicated, to determine its extent with biopsy 3
Treatment and Management
- Supportive treatment is the primary intervention, as HSP spontaneously resolves in 94% of children and 89% of adults 2, 3
- Oral prednisone at 1-2 mg per kg daily for two weeks has been used to treat abdominal and joint symptoms 2
- 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
Prognosis and Complications
- Long-term prognosis depends on the severity of renal involvement 2, 3
- End-stage renal disease occurs in 1-5% of patients 2
- Complications can include gastrointestinal bleeding, orchitis, and central nervous system involvement 3
- Six months of follow-up is prudent to assess for disease relapse or remission 3