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.