Successful treatment of IgG4-related hypertrophic pachymeningitis with induction rituximab and dexamethasone followed by maintenance rituximab.

IgG4-related disease (IgG4RD) with intracranial involvement is rare. We report a 56-year-old male who had an excellent response to rituximab and dexamethasone after going undiagnosed for 5 years. After 3 years of rituximab maintenance, he has no evidence of disease on brain MRI.