Rituximab is NOT Recommended for IgA Vasculitis
Rituximab has no established role in the treatment of IgA vasculitis (Henoch-Schönlein purpura), as current guidelines specifically address ANCA-associated vasculitis, which is a completely different disease entity. 1
Critical Distinction Between Disease Entities
- IgA vasculitis and ANCA-associated vasculitis are separate conditions with different pathophysiology, requiring distinct treatment approaches 1
- The KDIGO 2024 and EULAR/ERA-EDTA guidelines explicitly focus on ANCA-associated vasculitis (granulomatosis with polyangiitis and microscopic polyangiitis), not IgA vasculitis 2, 1
- No guideline-based recommendations exist for rituximab use in IgA vasculitis 1
Evidence Limitations
While one small case series from 2020 reported rituximab use in 12 adults with severe IgA vasculitis and crescentic nephritis, showing 91.7% clinical response at 6 months 3, this represents:
- Low-quality evidence: Single-center, uncontrolled case series without randomized comparison
- Highly selected population: Only refractory cases or patients with contraindications to standard therapy 3
- No guideline endorsement: This isolated report has not been incorporated into any clinical practice guidelines 1
Standard Treatment Approach for IgA Vasculitis
For IgA vasculitis with severe renal involvement, established treatments include:
- Corticosteroids as first-line therapy for significant renal disease
- Cyclophosphamide or mycophenolate mofetil for severe crescentic glomerulonephritis
- Supportive care for mild cases without organ-threatening manifestations
Common Pitfall to Avoid
Do not extrapolate ANCA-vasculitis treatment protocols to IgA vasculitis. Despite both being small-vessel vasculitides, they have fundamentally different immune mechanisms (ANCA-mediated vs. IgA immune complex-mediated) and require different therapeutic strategies 1.
When Rituximab Might Be Considered
Only in exceptional circumstances of truly refractory IgA vasculitis with life-threatening manifestations unresponsive to all standard therapies should rituximab be considered, and this should occur:
- At expert referral centers with experience in complex vasculitis management
- After documented failure of corticosteroids and conventional immunosuppressants
- With informed consent acknowledging off-label use without guideline support 3