Targeting the B Cell Lineage in IgG4-Related Disease



Despite the elevated serum concentration of IgG4 in many patients and the disproportionate number of IgG4+ plasma cells within the affected tissues that constitute essentially a sine qua non of IgG4-related disease, serum IgG4 itself appears to be a reactive phenomenon rather than the primary disease driver. Recent investigations have focused on the interactions between cells of the B cell lineage and a novel CD4+ cytotoxic T cell that is capable of promoting fibrosis.


Serum IgG4 Lacrimal Gland Cell Depletion Serum IgG4 Concentration Elevated Serum Concentration 


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Copyright information

© Springer Japan 2016

Authors and Affiliations

  1. 1.Rheumatology Clinic/Yawkey 2Massachusetts General HospitalBostonUSA

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