De novo oligoclonal expansions of circulating plasmablasts in active and relapsing IgG4-related disease.

ABSTRACT:

Background

IgG4-related disease (IgG4-RD) is a poorly understood, multiorgan, chronic inflammatory disease characterized by tumefactive lesions, storiform fibrosis, obliterative phlebitis, and accumulation of IgG4-expressing plasma cells at disease sites.

Objective

The role of B cells and IgG4 antibodies in IgG4-RD pathogenesis is not well defined. We evaluated patients with IgG4-RD for activated B cells in both disease lesions and peripheral blood and investigated their role in disease pathogenesis.

Methods

B-cell populations from the peripheral blood of 84 patients with active IgG4-RD were analyzed by using flow cytometry. The repertoire of B-cell populations was analyzed in a subset of patients by using next-generation sequencing. Fourteen of these patients were lo

SUBMITTER: Mattoo H 

PROVIDER: S-EPMC4149918 | biostudies-literature | 2014 Sep

REPOSITORIES: biostudies-literature