HYPERTROPHIC CRANIAL PACHYMENINGITIS IN MPO-ANCA-RELATED VASCULITIS: A CASE REPORT AND LITERATURE REVIEW

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  • WATANABE KIMIO
    Department of Internal Medicine, Division of Nephrology, Hypertension, Endocrinology, and Diabetology/Metabolism, Fukushima Medical University School of Medicine
  • TANI YOSHIHIRO
    Department of Internal Medicine, Division of Nephrology, Hypertension, Endocrinology, and Diabetology/Metabolism, Fukushima Medical University School of Medicine
  • KIMURA HIROSHI
    Department of Internal Medicine, Division of Nephrology, Hypertension, Endocrinology, and Diabetology/Metabolism, Fukushima Medical University School of Medicine
  • ASAI JUN
    Department of Internal Medicine, Division of Nephrology, Hypertension, Endocrinology, and Diabetology/Metabolism, Fukushima Medical University School of Medicine
  • TANAKA KENICHI
    Department of Internal Medicine, Division of Nephrology, Hypertension, Endocrinology, and Diabetology/Metabolism, Fukushima Medical University School of Medicine
  • HAYASHI YOSHIMITSU
    Department of Internal Medicine, Division of Nephrology, Hypertension, Endocrinology, and Diabetology/Metabolism, Fukushima Medical University School of Medicine
  • ASAHI KOICHI
    Department of Internal Medicine, Division of Nephrology, Hypertension, Endocrinology, and Diabetology/Metabolism, Fukushima Medical University School of Medicine
  • NAKAYAMA MASAAKI
    Department of Internal Medicine, Division of Nephrology, Hypertension, Endocrinology, and Diabetology/Metabolism, Fukushima Medical University School of Medicine
  • WATANABE TSUYOSHI
    Department of Internal Medicine, Division of Nephrology, Hypertension, Endocrinology, and Diabetology/Metabolism, Fukushima Medical University School of Medicine

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A 75-year-old woman presented with rapidly progressive glomerulonephritis with positive results for anti-myeloperoxidase anti-neutrophil cytoplasmic antibody (MPO-ANCA). Corticosteroid therapy was successfully introduced. However, 7 months later, magnetic resonance imaging revealed marked swelling in the falx cerebri and high density regions were apparent on gallium scintigraphy, leading to diagnosis of hypertrophic cranial pachymeningitis (HCP). Symptoms improved with intensified corticosteroid therapy, but radiological examination 9 months later revealed right nasal sinus inflammation accompanied by osteolytic change. Granulomatosis with polyangiitis (Wegener's) was finally diagnosed. HCP is an important complication in MPO-ANCA-related vasculitis, and needs to be considered during the clinical course.

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