The diagnosis of IgG4-related hypertrophic pachymeningitis is challenging but is of great relevance as treatment differs significantly from other forms of pachymeningitis and a specific therapeutic approach may avoid long-term neurological complications 1).
There is no specific treatment protocol for IgG4 related pachymeningitis 2).
High-dose steroids are the initial treatment of choice; other immunosuppressive agents have shown variable efficacy in reducing meningeal hypertrophy and mostly used in disease relapse setting 3)
Second-line is chemotherapy (methotrexate, azathioprine, etc.).
Surgery is necessary when lesions progress and patients start to develop cranial nerve function deficit 4).