ANCA-associated hypertrophic pachymeningitis, a central nervous system limited type of systemic vasculitis

Abstract

LEARNING POINT FOR CLINICIANS Hypertrophic pachymeningitis (HP) is characterized by a thickening of the dura mater that in some cases has been related to systemic diseases. Some hearing loss may be the first clinical manifestation of PH as a form of ANCA-positive vasculitis limited to the brain. Treatment with rituximab appears to be a good therapeutic option for these cases.