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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.
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.
Pre- and Post-treatment Serum BAFF Levels and BAFF Gene Polymorphisms in Patients with Graves’ Disease
Published in Endocrine Research, 2023
Tarak Dhaouadi, Imen Rojbi, Sameh Ghammouki, Ibtissem Ben Nacef, Meriem Adel, Sabrine Mekni, Karima Khiari, Taïeb Ben Abdallah, Imen Sfar, Yousr Gorgi
B cell activating factor (BAFF) is a critical factor for peripheral B cell maturation, survival, proliferation and differentiation in the periphery.4 In fact, either BAFF gene invalidation or in vivo neutralization prevents the persistence of mature B cells in periphery and impairs their ability to mount T-dependent and -independent immune responses.5 Besides, BAFF contributes to T cell proliferation and activation with cytokine production through BAFF-Receptor (BAFF-R) binding.6 Furthermore, BAFF is required for germinal center maintenance and plays a crucial role in the development of follicular helper T cells (Tfh) which are essential for somatic hypermutations and class switch recombination.6,7
Sjögren’s syndrome: shedding light on emerging and key drug targets
Published in Expert Opinion on Therapeutic Targets, 2022
Dominic Ridgewell, Nishanthi Thalayasingam, Wan-Fai Ng
Adaptive immunity also plays a key role in SS pathogenesis. B-cell dysfunction is a hallmark of SS pathology. BAFF (B-cell activating factor) provides a key link between innate immune responses and adaptive autoimmunity initiation, although other cytokines may also be involved [11,12]. BAFF levels are raised within the salivary glands of SS patients [10]. Serum BAFF levels correlate with an increase in the disease-associated autoantibodies anti-Ro/SSA, anti-La/SSB and rheumatoid factor (RF) [13] and clinical scores of disease activity [14]. In addition, BAFF transgenic mice develop a syndrome with clinical features similar to SS [15].