A novel model for treatment of hypertrophic pachymeningitis.

ABSTRACT: Objective:Immunoglobulin (Ig)G4-related disease is a major cause of hypertrophic pachymeningitis (HP), presenting as a progressive thickening of the dura mater. HP lacks an animal model to determine its underlying mechanisms. We developed a suitable animal model for the treatment of HP. Methods:We longitudinally evaluated dura in mice with a mutation (Y136F) in the linker for activation of T cells (LAT), which induced type 2 T helper (Th2) cell proliferation and IgG1 (IgG4 human equivalent) overexpression. Mice were therapeutically administered daily oral irbesartan from 3 to 6 weeks of age. Human IgG4-related, anti-neutrophil cytoplasmic antibody-related, and idiopathic HP dura were also immunohistochemically examined. Results:LATY136F mice showing dural gadolinium enhancement on magnetic

SUBMITTER: Cui Y 

PROVIDER: S-EPMC6414490 | biostudies-literature | 2019 Mar

REPOSITORIES: biostudies-literature