Association of BAFF/BLyS overexpression and altered B cell differentiation with Sjögren’s syndrome

BLyS and APRIL in rheumatoid arthritis

“…Such mice develop autoimmune-like manifestations reminiscent of human systemic lupus erythematosus (SLE) and Sjögren syndrome (SS) (19,20). Consistent with those data, elevated BLyS serum levels have been found in patients with RA, SLE, and SS (21)(22)(23)(24). In contrast, in BLyS -/-mice, B cell development in the spleen is impaired beyond the T1 transitional stage and in the peritoneum beyond the B1 stage.…”

“…BLyS serum levels are significantly increased in patients with RA (21), SLE (47), or SS (22). A recent report found elevated serum levels of APRIL in SLE patients compared with normal controls and patients with RA (48).…”

BAFF selectively enhances the survival of plasmablasts generated from human memory B cells

“…Patients with SLE exhibit elevated serum levels not only of BAFF (19)(20)(21), but also IL-10 aberrantly produced by B cells and monocytes (60). Because IL-10 has been found to be a potent inducer of BAFF production by human myeloid cells (55,56), it is possible that elevated levels of serum BAFF in SLE are secondary to the elevated levels of serum IL-10 in these patients.…”

B Lymphocyte Selection and Survival in Systemic Lupus

“…Patients with SLE also have increased levels of BAFF protein in their sera, and the titers of BAFF correlated well with the titers of anti-dsDNA autoantibody, but not with disease activity [136, 137]. Moreover, the biologic activity of circulating BAFF found in the sera of patients with SLE and Sjögren’s syndrome was higher than that in normal controls [137, 138, 139, 140, 141]. Nevertheless, there is a heterogeneity in BAFF levels among different lupus patients, and normal, or even lower than normal, levels of circulating serum BAFF are present in some patients [136].…”