A flare in mixed type II cryoglobulinemia vasculitis may occur following rituximab therapy.
Criteria for rituximab-associated flare: onset of new organ involvement or worsening of autoimmune disease <= 4 weeks after rituximab therapy (In many cases onset is less than 2 weeks after therapy).
This has been described in patients with mixed type II cryoglobulinemia vasculitis.
Clinical features:
(1) acute renal insufficiency
(2) purpura
(3) gastrointestinal involvement
(4) myocarditis
Laboratory features:
(1) increased levels of cryoglobulins (2.1 vs 0.4 g/L)
(2) decreased C4 complement
Renal biopsy shows glomerular endomembranous deposits that are positive for IgG1, IgM and rituximab and thrombi.
The flare is associated with increased mortality.
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