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As alluded to by Drs. Wasilewski and Samkoff,1 immunoglobulin G4 (IgG4)–related disease (RD) is an increasingly recognized cause of hypertrophic pachymeningitis (HP) and likely accounts for a substantial subset of cases previously thought to be idiopathic.2 Although normal IgG4 levels in serum and CSF argue against IgG4-related HP in this case, meningeal biopsy remains the diagnostic gold standard.3,4 Since IgG4-RD is a major consideration, it is of critical importance to immunostain biopsied tissue for the number of IgG4+ cells/high-powered field (HPF) prior to labeling the disease entity idiopathic; more than 10 IgG4+ cells/HPF suggests IgG4-related HP.4 Although both IgG4-related and idiopathic HP are inflammatory steroid-responsive illnesses, differentiation permits better understanding of individual disease mechanisms and may greatly help guide decision-making when selecting second-line therapies in treatment-resistant cases, such as the use of rituximab in refractory IgG4-related HP.5
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Author disclosures are available upon request (journal{at}neurology.org).
- © 2017 American Academy of Neurology
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