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Scleroderma
Published in Jason Liebowitz, Philip Seo, David Hellmann, Michael Zeide, Clinical Innovation in Rheumatology, 2023
Autoimmunity is a hallmark of SSc pathogenesis, and older studies have shown that SSc autoantibodies could also have functional properties, such as the pro-fibrotic effects of anti-PDGF-R antibodies (109). Defective B lymphocyte responses in patients with SSc have also been reported to contribute to increased production of key cytokines, such as IL-6 and IL-8 (110). Serum levels of BAFF (B cell activating factor), known to be a mediator of the maturation of B cells, have also been found to be elevated in SSc patients (111).
Monocyte and lymphocyte membrane markers: Ontogeny and clinical significance
Published in Gabriel Virella, Medical Immunology, 2019
Scott Sugden, Damien Montamat-Sicotte, Karen K. Yam, Joseph Murphy, Bader Yassine Diab, Virginia Litwin
Three cellular receptors of the TNFR family are involved in proliferation and maturation of B cells: the B cell maturation antigen (BCMA), the transmembrane activator and calcium modulator and cyclophilin ligand interactor (TACI), and the BLyS receptor 3 (BR3). BAFF (for B-cell activating factor) and a proliferation inducing ligand (APRIL) are soluble ligands belonging to a TNF subfamily that interacts with those receptors.
Pathogenesis
Published in Aparna Palit, Arun C. Inamadar, Systemic Sclerosis, 2019
Serum-light chain immunoglobulin (sFLC) levels are markers of polyclonal B cell activation. These levels are associated with the presence and severity of skin and lung fibrosis. Increased levels of B-cell activating factor (BAFF) in the sera and skin of patients with SSc indicate B cell activation and a degree of skin involvement. Along with β-microglobulin, BAFF correlates with the severity and activity of SSc.3 The increased expression of BAFF on B cells boosts their ability to produce IL-6 and immunoglobulins. BAFF, a potent B cell survival factor, plays an important role in the abnormal functioning of B cells. The serum level of BAFF is increased in patients with SSc and correlates with severity of SSc.
Biomarkers of lymphoma in Sjögren’s syndrome: what’s the latest?
Published in Expert Review of Clinical Immunology, 2022
Ioanna E. Stergiou, Athanasios-Dimitrios Bakasis, Stavroula Giannouli, Michael Voulgarelis
B cell activating factor (BAFF) is an essential factor for B-cell activation and proliferation, potentially involved in pSS-related B-cell deregulation [106]. Persistent B-cell activation, driven by increased BAFF levels, may contribute to lymphomagenesis [107,108]. Quartuccio et al. documented higher BAFF levels in pSS patients with lymphoma [109]. Interestingly, clonal B-cell expansion, but not polyclonal B-cell proliferation, was characterized by increased BAFF levels, thus making this biomarker ideal for identifying high-risk patients even at pre-lymphomatous stages [109]. The prospective study by Nocturne et al. confirmed that high BAFF levels are associated with NHL [77]. Gottenberg et al. demonstrated that pSS-lymphoma patients exhibit high BAFF serum levels even after lymphoma treatment and remission [79]. This finding suggests a possible genetic origin of such persistently increased BAFF levels.
Shedding light on developmental drugs for idiopathic pulmonary fibrosis
Published in Expert Opinion on Investigational Drugs, 2020
Paolo Spagnolo, Francesco Bonella, Christopher J Ryerson, Argyris Tzouvelekis, Toby M Maher
Several lines of evidence suggest a potential role for humoral response in the development and progression of IPF. B cell-rich lymphocyte aggregates are a common finding in end-stage IPF lung tissue [54], and levels of anti-vimentin autoantibodies inversely correlate with lung function parameters [55]. In addition, detection of B-cell-activating factor (BAFF) is enriched in the lungs and blood of patients with IPF [56]. Furthermore, rituximab, a B-lymphocyte depleting monoclonal antibody, has been used successfully as a rescue therapy in patients with severe ILD progressing despite conventional treatment, although this retrospective study did not include patients with IPF [57]. VAY736/ianalumab is an IgG1 monoclonal antibody targeting BAFF-R that is being developed for the treatment of several immune-mediated conditions, including autoimmune hepatitis, rheumatoid arthritis, Sjögren’s syndrome, and systemic lupus erythematosus. An ongoing phase II trial will evaluate the safety and efficacy of VAY736/ianalumab administered subcutaneously every 4 weeks for 48 weeks in patients with IPF (ClinicalTrial.gov Identifier: NCT03287414).
Current pharmacotherapeutic options for myasthenia gravis
Published in Expert Opinion on Pharmacotherapy, 2019
Carolina Barnett, Raha Tabasinejad, Vera Bril
B-cell activating factor (BAFF) is a cytokine leading to the differentiation of B cells into antibody-secreting cells [68]. Belimumab is a humanized anti-BAFF monoclonal antibody that interferes with the binding of BAFF to its B cell receptors and therefore interferes with differentiation of B cells and presumably antibody secretion. Disappointingly, anti-B cell therapy with Belimumab proved ineffective in improving MG as primary therapy or adjunctive therapy [69]. Belimumab failed to demonstrate any significant effect on QMGS when added to current standard-of-care treatment for 24 weeks in 40 patients with generalized MG in a phase II placebo-controlled study [69]. A higher proportion of patients on belimumab tended to improve than on placebo but the difference was not significant. It may be that the MG was too mild in this patient group who had a median QMGS of 12 on entering the study.