Background/Purpose: Mixed cryoglobulinemia syndrome (MCs) is a systemic vasculitis characterized by multiple organ involvement due to the vascular deposition of immune-complexes, mainly the cryoglobulins. B cells expansion frequently triggered by HCV infection plays a central role in MCs. The long term effects of B-cells depletion in MCs are still on debate
Methods:
Twenty seven patients, (mean age 60.2 [range 35-78] years, HCV infection in 96% of cases) with symptomatic type-II MCs with systemic manifestations, including renal involvement (diffuse membranoproliferative glomerulonephritis in 15 cases), peripheral neuropathy (26 cases) and large skin ulcers (9 case, in 7 necrotizing) were considered eligible for Rituximab (RTX) therapy. RTX was administered at a dose of 375 mg/m2 on days 1, 8, 15 and 22. Two more doses were administered 1 and 2 months later. No other immunosuppressive drugs were added. Response was evaluated by assessing the changes in clinical signs, symptoms, laboratory parameters and electromyographic indices for a very long term follow-up (mean 54.3 months [12-96])
Results: Complete remission of pre-treatment active manifestations was observed in all the cases of skin purpuric lesions and non-healing vasculitic leg ulcers, and in 80% of the peripheral neuropathy, mainly paresthesias. A significant improvement in the clinical neuropathy disability score was observed. Electromyography examination revealed that the amplitude of compound motor action potential had increased. Cryoglobulinemic nephropathy, observed in 15 patients, significantly improved during the follow-up starting from the second month after RTX (serum creatinine from 2.2±1.9SD to 1.6±1.2SD mg/dl, p≤.05; 24-hour proteinuria from 2.3±2.1SD to 0.9±1.9SD g/24h, p≤.05). Improvement of cryoglobulinemic serological hallmarks, such as cryocrit and low complement C4, were reported. The safety of RTX was confirmed by the absence of side effects recorded during the mean 54-month follow-up. Re-induction was performed in 9 relapsed cases (after a mean of 31.1 months, range 12-54) with resolutive beneficial effects
Conclusion:
In this open prospective study, RTX appeared to be effective and safe in the treatment of patients with MCs-associated neuropathy and membranoproliferative nephritis
Disclosure:
D. Roccatello,
None;
S. Sciascia,
None;
S. Baldovino,
None;
D. Rossi,
None.
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ACR Meeting Abstracts - https://acrabstracts.org/abstract/a-4-plus-2-infusion-protocol-of-rituximab-provides-long-term-beneficial-effects-in-patients-with-hcv-associated-mixed-cryoglobulinemia-with-membranoproliferative-nephritis-and-severe-polyneuropathy/