Abstract
To evaluate the efficacy and safety in the long term of a retreatment regimen with Rituximab (RTX) alone administered at clinical relapse in cryoglobulinemic vasculitis (CV). Thirty patients with severe HCV-related CV, previously enrolled in the multicentre Italian trial on RTX in the treatment of CV, were retrospectively evaluated after the end of the trial. All of them were managed with RTX alone at clinical relapse, if any. Disease activity at the last available follow up was defined as complete remission (absence of active disease), partial remission (response > 50% of at least one manifestation among glomerulonephritis, peripheral neuropathy or skin ulcers) or active disease. The mean follow up after the first RTX cycle was 72.6 (20.4) months. After the end of the trial, 21/30 (70%) patients showed an active follow up [81.7 (10.9) months)], 3/30 (10%) lost follow up and 6/30 (20%) died. 12/21 (57.1%) patients were in complete disease remission, 5/21 (23.8%) showed a partial response and 4/21 (19%) had an active disease. 17/30 (56.7%) patients needed retreatment for relapse with a mean time to retreatment of 22.3 (12.1) months. Treatment survival of this regimen was 7.6 (0.3) years. Recurrent non-severe infections occurred in 3/30, with chronic hypogammaglobulinemia in 2/3 patients. A long-term regimen of retreatment with RTX alone given at clinical relapse seems to be effective and safe in CV, with a low rate of infections and severe hypogammaglobulinemia.
Highlights
Cryoglobulinemic vasculitis (CV) is a systemic vasculitis, usually triggered by HCV infection [1], and characterized by an expansion of oligo-monoclonal B cells that produce IgM with rheumatoid factor (RF) activity, which can lead to the formation of immune complexes consisting of RF, HCV and polyclonal HCV-specific IgG, precipitating in blood vessel walls or glomerular capillaries
Patients were categorized into three groups, based on the response to RTX evaluated at the last available follow-up visit, as in complete remission, partial response or active disease
The mean survival time of the whole RTX treatment was 7.6 (0.3) years; as shown in Fig. 1, this survival was slightly longer for nephritis [7.9 (0.4) years] than for neuropathy [6.7 (0.4) years]; while it could be not evaluated in the subgroup of patients where the indication to retreatment were the skin ulcers, since only 5 patients were included
Summary
Cryoglobulinemic vasculitis (CV) is a systemic vasculitis, usually triggered by HCV infection [1], and characterized by an expansion of oligo-monoclonal B cells that produce IgM with rheumatoid factor (RF) activity, which can lead to the formation of immune complexes consisting of RF, HCV and polyclonal HCV-specific IgG, precipitating in blood vessel walls or glomerular capillaries. CV is characterized by the typical clinical triad of purpura, weakness, and arthralgia and often by severe organ involvement including glomerulonephritis, peripheral neuropathy, and skin ulcer [2]. Treatment of HCV-related CV may target either the viral trigger or the downstream B cell arm of autoimmunity. The new direct acting antiviral drugs for HCV are being investigated in HCV-related CV. Antiviral treatment is presently not the first choice, based on clinical priorities, in HCVpositive patients with severe CV organ involvement [3e8]
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