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Washington University Experience | MISCELLANEOUS | Idiopathic Hypertrophic Pachymeningitis (IgG4 Disease) | 8I2 Brain (Case 8) A2 CD163 20X 5
The granulomas surrounding vessels are strongly CD163 immunoreactive. Giant cells are not a part of these structures in this case. (CD163 IHC) ---- Comment: The collective findings are consistent with dura mater and leptomeninges with granulomatous inflammation, small vessel vasculopathy/vasculitis and increased IgG4+ plasma cells. We have seen several of these cases, some with circulating antibody excess, often ANCA. Our current case has normal serum IgG4 and no evidence of ANCA, not unusual in our experience. There is no evidence of organisms, either in the mastoid and intracranial specimens or by culture. Small vessel vasculitis and IgG4 excess suggests a vasculitis/IgG4 overlap syndrome (PMID: 28780079)
