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Pathophysiology of IgG4-related disease: A T follicular helper cells disease?
Benjamin De Sainte Marie1, Maria Laetizia Urban1, Frédéric Vély2
1Département de médecine interne, Hôpital de la Timone, AP-HM, Aix-Marseille Université, 13005 Marseille, France.
Abstract:
IgG4-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.
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