Rituximab
Anti-CD20 monoclonal antibody; depletes B cells
- Response rate
- Promising in refractory cases
- Onset
- Weeks–months
- Route
- IV 375 mg/m² x4 or 1000 mg x2
- Line
- 3rd
- IgM effect
- Reduces anti-C1q antibody production via B-cell depletion
Evidence summary
B-cell depletion with rituximab reduces anti-C1q autoantibody production. Shown effective in refractory HUVS, especially with renal involvement. Long-term follow-up of pediatric DNASE1L3-mutant siblings showed rituximab was the most effective treatment. Emerging as a key option for severe/refractory disease.