IgA Vasculitis: Clinical Manifestations and Treatment
Clinical Symptoms
IgA vasculitis (IgAV), formerly Henoch-Schönlein purpura, presents with four cardinal features: palpable purpura, arthralgia/arthritis, abdominal pain, and glomerulonephritis. 1
Cutaneous Manifestations
- Round or oval palpable purpura predominantly affecting the lower legs and buttocks, which may progress to retiform patterns 1
- The purpuric rash is the hallmark feature and results from leukocytoclastic vasculitis with perivascular IgA deposition 1
Musculoskeletal Symptoms
- Arthralgia or frank arthritis, typically affecting large joints (knees, ankles) 1, 2
- Joint involvement is usually self-limited and non-deforming 1
Gastrointestinal Manifestations
- Colicky abdominal pain is the predominant GI symptom 1, 2
- Gastrointestinal bleeding presenting as hematemesis or hematochezia 2
- Severe complications can include intestinal ischemia, though this is rare 3
- GI symptoms may be complicated by concurrent infections (bacterial, viral, or parasitic) that can trigger disease flares 2
Renal Involvement
- Glomerulonephritis with mesangial IgA deposits (IgAV nephritis) 1, 4
- Presents with hematuria and proteinuria 1
- Adults have significantly worse renal prognosis than children, with up to one-third progressing to end-stage renal failure 4
Less Common Manifestations
Treatment Algorithm
Mild, Self-Limited Disease
For isolated cutaneous and mild joint symptoms without organ-threatening features, symptomatic treatment alone is recommended. 1, 4
- Supportive care with analgesics and rest 1
- No immunosuppression required for self-limited disease 1, 4
Severe Cutaneous Disease with Incipient Necrosis
Initiate glucocorticoids at prednisolone 1 mg/kg/day (maximum 60-80 mg/day) for severe skin manifestations with incipient necrosis. 5
- Taper to maintenance dose of 10 mg/day or less during remission 5
- Gradual reduction after 6-18 months depending on response 5
Severe Gastrointestinal Complications
Start high-dose corticosteroids (prednisolone 1 mg/kg/day, maximum 60-80 mg/day) for severe GI manifestations including bleeding or severe pain. 5, 2
- Treatment with proton pump inhibitors for GI bleeding 2
- Screen for concurrent GI infections (bacterial, viral, parasitic) as these can complicate disease course 2
- Intravenous fluids for volume resuscitation if significant bleeding 2
Severe Renal Involvement (Proliferative Glomerulonephritis)
Combine glucocorticoids with immunosuppressive therapy for proliferative glomerulonephritis or IgAV nephritis with risk factors for progression. 5
Induction Options:
- Cyclophosphamide combined with glucocorticoids 5, 6
- Rituximab combined with glucocorticoids 5, 6
- Mycophenolate mofetil combined with glucocorticoids 5, 6
Maintenance Options:
- Calcineurin inhibitors (cyclosporine A or tacrolimus) as glucocorticoid-sparing agents 5
- Mycophenolate mofetil for maintenance 5
Refractory or Life-Threatening Disease
Consider plasma exchange therapy combined with immunosuppression for patients failing to achieve remission or with life-threatening manifestations. 5, 3
- Plasma exchange has shown efficacy in refractory cases with ischemic complications (renal infarction, intestinal ischemia) 3
- Intravenous immunoglobulin (IVIG) can be used for refractory cases 5, 3
- Critical caveat: Measure serum immunoglobulin levels before IVIG to avoid anaphylaxis in patients with selective IgA deficiency 5
Key Clinical Distinctions: Adults vs. Children
Adult-onset IgAV has a fundamentally different prognosis than pediatric disease, with more severe and frequent nephritis. 1, 4
- Adults develop IgAV 150-200 times less frequently than children (incidence 1 in 1 million/year) 1, 4
- Up to one-third of adults progress to end-stage renal failure, compared to much lower rates in children 4
- Short-term outcome depends on severity of GI manifestations 1, 4
- Long-term prognosis is determined by presence and severity of nephritis 1, 4
Critical Pitfalls to Avoid
Do Not Confuse with ANCA-Associated Vasculitis
- IgAV is not ANCA-associated vasculitis and should not be treated with the intensive protocols used for granulomatosis with polyangiitis or microscopic polyangiitis 5
- The evidence provided for ANCA vasculitis management 7 does not apply to IgAV 5
Monitor for Infectious Complications
- GI infections (Norovirus, bacterial, parasitic) can complicate IgAV and trigger severe symptoms or relapses 2
- Screen stool for pathogens in patients with severe GI symptoms 2