Management of IgA Vasculitis
For IgA vasculitis, treatment should be stratified by disease severity: supportive care alone for mild self-limited disease, glucocorticoids (prednisolone 1 mg/kg/day, maximum 60-80 mg/day) for severe skin manifestations with incipient necrosis or severe gastrointestinal complications, and combination immunosuppression with glucocorticoids plus cyclophosphamide, rituximab, or mycophenolate mofetil for proliferative glomerulonephritis or nephritis with risk factors for progression. 1
Disease Severity Stratification
Mild, Self-Limited Disease
- Supportive care alone is sufficient for uncomplicated cutaneous purpura, mild arthralgias, and minor gastrointestinal symptoms without bleeding or severe pain. 2, 3
- This approach is particularly appropriate in children, where the disease course is typically benign and self-limiting. 4, 2
- Adults require closer monitoring as they experience more severe disease and higher rates of renal involvement compared to children (150-200:1 ratio). 4, 3
Severe Cutaneous or Gastrointestinal Manifestations
- Initiate glucocorticoids at prednisolone 1 mg/kg/day (maximum 60-80 mg/day) for severe skin manifestations with incipient necrosis or severe gastrointestinal complications including hematemesis, hematochezia, or severe colicky abdominal pain. 1
- Taper glucocorticoids to a maintenance dose of 10 mg/day or less during remission, with gradual reduction after 6-18 months depending on response. 1
- Add proton pump inhibitors and intravenous fluids for gastrointestinal bleeding. 5
Critical pitfall: Severe gastrointestinal symptoms may be complicated by concomitant infections (42.9% bacterial, 25.7% viral, 17.1% parasitic), requiring stool analysis and pathogen-directed therapy in addition to immunosuppression. 5
Management of Renal Involvement
Proliferative Glomerulonephritis or High-Risk IgA Vasculitis Nephritis
- Combine glucocorticoids with immunosuppressive therapy—cyclophosphamide, rituximab, or mycophenolate mofetil—for proliferative glomerulonephritis or IgAV nephritis with risk factors for progression (baseline proteinuria >1-1.5 g/day, baseline renal impairment, or biopsy showing interstitial fibrosis, sclerotic glomeruli, or fibrinoid necrosis). 1, 6
- Consider calcineurin inhibitors (cyclosporine A or tacrolimus) or mycophenolate mofetil as glucocorticoid-sparing maintenance agents. 1
Key prognostic factors: Up to one-third of adult patients with nephritis progress to end-stage renal disease, making aggressive treatment of high-risk features essential. 4, 6
Refractory or Life-Threatening Disease
- Consider plasma exchange therapy combined with immunosuppression for patients failing to achieve remission or with life-threatening manifestations. 1
- Intravenous immunoglobulin (IVIG) may be used for refractory cases, but measure serum immunoglobulin levels first to avoid anaphylaxis in patients with selective IgA deficiency. 1
Monitoring and Follow-Up
- Long-term follow-up is mandatory as prognosis depends heavily on the presence and severity of nephritis, with renal outcomes similar to IgA nephropathy. 4, 3
- Monitor for gastrointestinal infections in patients with severe abdominal symptoms, as these can cause significant morbidity and mortality, especially in adults. 5
- Short-term outcome depends on severity of gastrointestinal manifestations, while long-term prognosis is determined by nephritis severity. 3
Important caveat: The absence of correlation between initial presentation and long-term renal outcome makes risk stratification challenging—patients with mild symptoms may progress to ESRD while those with severe presentation may experience spontaneous remission. 6 This unpredictability mandates close monitoring regardless of initial severity.