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 journalArticle

2 Citations (Scopus)

Abstract

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)
Volume97
Issue number17
DOIs
Publication statusPublished - 2018 Apr 1

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Myeloblastin
Antineutrophil Cytoplasmic Antibodies
Meningitis
Immunosuppressive Agents
Prednisolone
Mastoiditis
Therapeutics
Cranial Nerve Diseases
Dysarthria
Temporal Bone
Methotrexate
C-Reactive Protein
Cyclophosphamide
Ear
Headache
Fibrosis
Steroids
Tomography
Magnetic Resonance Imaging
Anti-Bacterial Agents

Keywords

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

ASJC Scopus subject areas

  • Medicine(all)

Cite this

Adolescent PR3-ANCA-positive hypertrophic pachymeningitis. / Matsumoto, Kotaro; Akiyama, Mitsuhiro; Kajio, Nobuhiko; Otomo, Kotaro; Suzuki, Kazuko; Nishina, Naoshi; Kasuya, Kento; Oishi, Naoki; Kameyama, Kaori; Takeuchi, Tsutomu.

In: Medicine (United States), Vol. 97, No. 17, e0521, 01.04.2018.

Research output: Contribution to journalArticle

Matsumoto, Kotaro ; Akiyama, Mitsuhiro ; Kajio, Nobuhiko ; Otomo, Kotaro ; Suzuki, Kazuko ; Nishina, Naoshi ; Kasuya, Kento ; Oishi, Naoki ; Kameyama, Kaori ; Takeuchi, Tsutomu. / Adolescent PR3-ANCA-positive hypertrophic pachymeningitis. In: Medicine (United States). 2018 ; Vol. 97, No. 17.
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AB - 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.

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