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Primary Sjögren syndrome (pSS) may be complicated by type II cryoglobulinaemia in approximately 5%–20% of patients. pSS is actually the first aetiology of type II cryoglobulinaemia,1 the latter being associated with development of vasculitis, extraglandular involvement, increased risk of B-cell lymphoma and decrease in survival.
Rituximab, a monoclonal anti-CD20 antibody, is frequently used in the treatment of cryoglobulinaemia. B-cell activating factor (BAFF), also known as B-lymphocyte stimulator, plays a key role in the survival and activation of B cells. An elevation in BAFF levels occurs after B-cell depletion induced by rituximab.2 It is the consequence of a decrease in B cells that are the most important reservoir of cells expressing the BAFF receptor and of an upregulation of BAFF mRNA.3 The association of belimumab with rituximab could be synergistic and is currently evaluated in pSS and systemic lupus erythematosus.4
We report three cases of patients with refractory cryoglobulinaemic vasculitis complicating pSS successfully treated by the combination of an anti-CD20 therapy followed by belimumab (table 1, …
XM and GN are joint senior authors.
Handling editor Josef S Smolen
Contributors KC and GN wrote the manuscript. RB and RS reviewed and corrected the manuscript. XM reviewed, corrected the manuscript and helped with the submission.
Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.
Competing interests None declared.
Patient and public involvement Patients and/or the public were not involved in the design, or conduct, or reporting, or dissemination plans of this research.
Patient consent for publication Not required.
Provenance and peer review Not commissioned; externally peer reviewed.
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