AI Article Synopsis
- Acquired C1-inhibitor deficiency can be caused by types I and II, with type II associated with anti-C1-inhibitor autoantibodies.
- Efficacy of treatments like danazol and corticosteroids varies, but rituximab shows some promise in type II angioedema.
- In a study of 7 patients treated with rituximab, type II showed mixed results, with only a subset achieving significant improvement and indicating that multiple treatments may be necessary.
Article Abstract
Background: Acquired C1-inhibitor deficiency can occur secondary to excessive C1-inhibitor consumption (type I) and be associated with a lymphoid hemopathy, or linked to the presence of anti-C1-inhibitor autoantibodies (type II) in a context of an isolated monoclonal gammopathy, sometimes associated with lymphoproliferation. Efficacy of danazol, tranexamic acid and/or corticosteroids is inconstant. Rituximab efficacy against type II angioedema has been reported.
Methods: Description of 7 rituximab-treated patients, 6 with type II acquired angioedema and 1 with type I.
Results: Clinical efficacy (only for type II) was complete for 3, partial for 2 and 2 were therapeutic failures. Only 2 patients had improved biological parameters, with normalization of their C1-inhibitor levels and diminished anti-C1-inhibitor autoantibodies, observed 1-9 months after the last infusion of the second rituximab cycle. An associated lymphoproliferation did not affect the response to treatment.
Conclusion: Rituximab efficacy in the treatment of acquired angioedema is inconstant and might require repeated cycles.
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| http://dx.doi.org/10.1007/s10875-012-9691-2 | DOI Listing |
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