Abstract
IntroductionB cell-activating factor of the tumor necrosis factor family (BAFF) and a proliferation-inducing ligand (APRIL) play a crucial role in B cell development, survival, and antibody production. Here we analyzed the serum levels of BAFF and APRIL and their respective clinical associations in patients with an immunoglobulin (Ig) G4-related disease (IgG4-RD).MethodsWe measured serum levels of BAFF and APRIL in patients with IgG4-RD, primary Sjögren's syndrome (pSS), and healthy individuals. Serum BAFF and APRIL levels in IgG4-RD were assessed for correlations with serological parameters, including Ig, particularly IgG4, and the number of affected organs. Serum BAFF and APRIL levels in IgG4-RD were monitored during glucocorticoid (GC) therapy.ResultsSerum BAFF and APRIL levels in patients with IgG4-RD were significantly higher (P < 0.01) than in healthy individuals. The BAFF levels of patients with IgG4-RD were comparable to those of patients with pSS. Although clinical parameters, such as serum IgG4 and the number of affected organs, were not correlated with the levels of BAFF, serum APRIL levels were inversely correlated with serum IgG4 levels (r = -0.626, P < 0.05). While serum BAFF levels decreased following GC therapy, serum APRIL levels increased during follow-up.ConclusionThese results indicate that BAFF and APRIL might be useful markers for predicting disease activity in IgG4-RD. Further studies are needed to elucidate the role of BAFF and APRIL in the pathogenesis of IgG4-RD.
Highlights
B cell-activating factor of the tumor necrosis factor family (BAFF) and a proliferation-inducing ligand (APRIL) play a crucial role in B cell development, survival, and antibody production
While serum BAFF levels decreased following GC therapy, serum APRIL levels increased during follow-up
BAFF binds to three receptors - BAFF receptor (BAFF-R), transmembrane activator and calcium-modulating cyclophilin ligand interactor (TACI), and B cell maturation antigen (BCMA) - which are expressed by B cells, whereas APRIL binds to TACI and BCMA [25]
Summary
B cell-activating factor of the tumor necrosis factor family (BAFF) and a proliferation-inducing ligand (APRIL) play a crucial role in B cell development, survival, and antibody production. B cell-activating factor of the tumor necrosis factor (TNF) family (BAFF, known as B-lymphocyte stimulator (BLyS) or TNF and apoptosis leukocyteexpressed ligand-1 (TALL-1)) and its homolog, a proliferation-inducing ligand (APRIL, known as TNFrelated death ligand 1 (TRDL-1) or TNF and apoptosis leukocyte-expressed ligand-2 (TALL-2), are members of the trimeric TNF family, and both play an essential role in the homeostasis of peripheral B cells [22]. Both cytokines are known to be expressed by a variety of cell types, the myeloid-lineage cells [22,23]. BAFF binds to three receptors - BAFF receptor (BAFF-R), transmembrane activator and calcium-modulating cyclophilin ligand interactor (TACI), and B cell maturation antigen (BCMA) - which are expressed by B cells, whereas APRIL binds to TACI and BCMA [25]
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