The B-cells paradigm in Systemic Sclerosis: an update on pathophysiology and B-cell targeted therapies.

Autor:	Scaletti C; Department of Experimental and Clinical Medicine, University of Florence, Florence, Italy., Pratesi S; Flow Cytometry Diagnostic Center and Immunotherapy, University Hospital Careggi, Florence, Italy., Bellando Randone S; Department of Clinical and Experimental Medicine, Rheumatology Unit, University of Florence, and Scleroderma Unit, University Hospital Careggi, Florence, Italy., Di Pietro L; Department of Experimental and Clinical Medicine, University of Florence, Florence, Italy., Campochiaro C; Unit of Immunology, Rheumatology, Allergy and Rare diseases (UnIRAR), IRCCS San Raffaele Hospital, Milan, Italy., Annunziato F; Department of Experimental and Clinical Medicine, University of Florence, Florence, Italy.; Flow Cytometry Diagnostic Center and Immunotherapy, University Hospital Careggi, Florence, Italy., Matucci Cerinic M; Unit of Immunology, Rheumatology, Allergy and Rare diseases (UnIRAR), IRCCS San Raffaele Hospital, Milan, Italy.; Vita Salute San Raffaele, University, Milan, Italy.
Jazyk:	angličtina
Zdroj:	Clinical and experimental immunology [Clin Exp Immunol] 2024 Nov 05. Date of Electronic Publication: 2024 Nov 05.
DOI:	10.1093/cei/uxae098
Abstrakt:	Systemic sclerosis is considered a rare autoimmune disease in which there are alterations of both the innate and adaptive immune response resulting in the production of autoantibodies. Abnormalities of the immune system compromise the normal function of blood vessels leading to a vasculopathy manifested by Raynaud's phenomenon, an early sign of systemic sclerosis. As a consequence of this reactive picture, the disease can evolve leading to tissue fibrosis. Several systemic sclerosis-specific autoantibodies are currently known and are associated with specific clinical manifestations and prognosis. Although the pathogenetic role of these autoantibodies is still unclear, their production by B cells and plasma cells suggests the importance of these cells in the development of systemic sclerosis. This review narratively examines B cell dysfunctions and their role in the pathogenesis of systemic sclerosis and discusses B cell-targeted therapies currently used or potentially useful for the management of end-organ complications. (© The Author(s) 2024. Published by Oxford University Press on behalf of the British Society for Immunology.)
Databáze:	MEDLINE
Externí odkaz:	Zobrazit plný text záznamu