IgG4-related disease is now recognised as an important cause of intracranial and spinal hypertrophic pachymeningitis. Treatment with corticosteroids generally leads to significant clinical improvement. We present two cases of IgG4 pachymeningitis unresponsive to corticosteroids who improved with rituximab.
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Contributors AM and RD were responsible for the planning, conducting and reporting of the study. NS, MF and AL were responsible for the reporting. JA, HA-B and CR were involved in the conducting. OG did the planning.
Competing interests None declared.
Patient consent Obtained.
Provenance and peer review Not commissioned; externally peer reviewed. This paper was reviewed by Neil Scolding, Bristol, UK.