Adolescent PR3-ANCA-positive hypertrophic pachymeningitis

Kotaro Matsumoto, Mitsuhiro Akiyama, Nobuhiko Kajio, Kotaro Otomo, Kazuko Suzuki, Naoshi Nishina, Kento Kasuya, Naoki Oishi, Kaori Kameyama, Tsutomu Takeuchi

Research output: Contribution to journalArticlepeer-review

12 Citations (Scopus)


Rationale: Hypertrophic pachymeningitis (HP) is an uncommon, life-threatening disease that is seen in elderly patients with antineutrophil cytoplasmic antibody (ANCA) positivity. Proteinase-3 (PR3)-ANCA-positive HP has not been reported in adolescents. Here, we report the first case of adolescent PR3-ANCA-positive HP successfully treated with immunosuppressive therapy. Patient concerns: A 14-year-old female presented with fullness and pain in her right ear unresponsive to antibiotics. Laboratory tests showed an elevated C-reactive protein and PR3-ANCA positivity. Computed tomography and magnetic resonance imaging revealed mastoiditis in the right temporal bone. Surgical biopsy revealed severe fibrosis and prominent inflammatory-cell infiltration. She received prednisolone and methotrexate therapy, and then underwent a right mastoidectomy. Five months later, she developed headache, dysarthria, and multiple cranial nerve palsies. Further imaging revealed enhancement and thickening of the right hemispheric dura. Diagnosis: PR3-ANCA-positive HP. Interventions: She was successfully treated with steroid pulse therapy for 3 days, followed by high doses of prednisolone and intravenous cyclophosphamide. Outcome: The treatment resulted in significant improvement of her symptoms, laboratory data, and radiologic findings. Lessons: PR3-ANCA-positive HP can present not only in the elderly, but also in adolescence, and prompt diagnosis and treatment with immunosuppressive therapy is vital.

Original languageEnglish
Article numbere0521
JournalMedicine (United States)
Issue number17
Publication statusPublished - 2018 Apr 1


  • adolescence
  • hypertrophic pachymeningitis (HP)
  • proteinase-3 anti-neutrophil cytoplasmic antibody (PR3-ANCA)

ASJC Scopus subject areas

  • General Medicine


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