Few data are available concerning pachymeningitis in GPA and other ANCA-associated vasculitis.[3,8–12] One of the recent important series analyzed central nervous system involvement in GPA and found a 46% prevalence of pachymeningitis, defining a granulomatous pattern.[11] These patients commonly have headaches and more frequently myeloperoxidase-ANCA, which differs from our series, 64% with PR3 specificity. This evidence concerns the gene MPO and granulomatosis with polyangiitis.