IntroductionAnti-Jo-1 and anti-Ro52 autoantibodies are common in patients with myositis, but the mechanisms for their production are not known. Survival of autoantibody producing cells is dependent on B cell activating factor of the TNF family (BAFF). BAFF levels are elevated in serum of anti-Jo-1+ myositis patients and are influenced by type I interferon (IFN). IFN producing cells and BAFF mRNA expression are present in myositis muscle. We investigated expression of the receptors for BAFF in muscle tissue in relation to anti-Jo-1 and anti-Ro52/anti-Ro60 autoantibodies and type I IFN markers.MethodsMuscle biopsies from 23 myositis patients, selected by autoantibody profile and seven healthy controls were investigated for expression of BAFF receptor (BAFF-R), B cell maturation antigen (BCMA) and transmembrane activator and calcium modulator and cyclophilin ligand interactor (TACI). Nineteen of these were assessed for plasma (CD138) and B cell (CD19) markers. Positively stained cells per area were compared with expression of plasmacytoid dendritic cell (pDC) marker blood dendritic cell antigen-2 (BDCA-2) and IFN¿/ß-inducible myxovirus resistance-1 protein (MX-1).ResultsBAFF-R, BCMA and TACI were expressed in five, seven and seven patients respectively and more frequently in anti-Jo-1+ and/or anti-Ro52/anti-Ro60+ patients compared to patients without these autoantibodies and controls (P¿=¿BAFF-R: 0.007, BCMA: 0.03 and TACI: 0.07). A local association of receptors with B and plasma cells in serial sections was confirmed by confocal microscopy. The numbers of CD138+ and BCMA+ cells correlated (rs¿=¿0.79; P¿=¿0.001).Expression of BDCA-2 correlated with numbers of CD138+ and marginally with BCMA+ cells (rs¿=¿0.54 and 0.42; P¿=¿0.04 and 0.06). There was a borderline correlation between the number of TACI+ cells and MX-1+ areas (rs¿=¿0.38; P¿=¿0.08).ConclusionsThe expression pattern of BAFF receptors on B and plasma cells in muscle suggests a local role for BAFF in autoantibody production in muscle tissue of myositis patients with anti-Jo-1 or anti-Ro52/anti-Ro60 autoantibodies. BAFF production could be influenced by type I IFN produced by pDCs. Thus B cell related molecular pathways may have a role in the pathogenesis in this subset of myositis patients.