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- Benjamin De Sainte Marie, Maria Laetizia Urban, Frédéric Vély, Julie Seguier, Aurélie Grados, Laurent Daniel, Mikael Ebbo, and Nicolas Schleinitz.
- Département de médecine interne, Hôpital de la Timone, AP-HM, Aix-Marseille Université, 13005 Marseille, France.
- Presse Med. 2020 Apr 1; 49 (1): 104013.
AbstractIgG4-related disease is a chronic inflammatory disease characterized by clinical, biological and pathological unifying findings. Because these criteria are not always all together available in patients and because biological and pathological markers are not totally specific, the diagnosis should be retained after exclusion of mimickers. Since the individualization of IgG4-RD, several studies have allowed to better characterize immunological abnormalities associated with this particular condition. B and T cell oligoclonal activation is associated with T helper 2 cytokine production leading to IgG4 production and profibrotic cytokine release. A central role for T follicular helper 2 cells is suggested from recent findings. We summarize here recent advances in understanding of immune abnormalities in IgG4-related disease.Copyright © 2020 Elsevier Masson SAS. All rights reserved.
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