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

Abstract

Abstract 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.

Keywords

• headache
• IgG4 disease
• lymphoproliferative disorder
• maintenance rituximab
• pachymeningitis