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, online supplementary table 1 and figure 1). Case 1, a 78-year-old woman, presented with pSS and refractory type II cryoglobulinaemia vasculitis with cutaneous and glomerular involvement despite corticosteroids, rituximab and cyclophosphamide, given successively and even in combination. After a new course of rituximab that failed to improve vasculitis and nephritis, a combination of rituximab (1 g) followed 1 week later by belimumab (200 mg/week) led to an improvement of renal involvement (creatininaemia= 81 μmol/L and proteinuria= 0.22 g/day) 13 weeks after the initiation of the treatment. EULAR Sjögren's syndrome disease