Severe destructive nasopharyngeal granulomatosis with polyangiitis with superimposed skull base Pseudomonas aeruginosa osteomyelitis

Mitchell S. Von Itzstein, Jithma P. Abeykoon, Daniel D. Summerfield, Jennifer A. Whitaker

Research output: Contribution to journalArticle

3 Citations (Scopus)

Abstract

Skull base osteomyelitis in the setting of granulomatosis with polyangiitis (GPA) is rare and entails significant diagnostic challenges. We present a case of a 65-year-old Caucasian man with a history of rheumatoid arthritis, off immunosuppression for 18 months, who presented with 2 years of chronic headaches, severe fatigue, saddle nose deformity and 20-kilogram unintentional weight loss. Maxillofacial CT revealed an extensive destructive sinonasal and erosive skull base process. Laboratory evaluation showed equivocal elevation of antiproteinase 3 antibodies with negative antineutrophil cytoplasmic antibody panel. Biopsy of the skull base/clivus revealed necrotising granulomatous inflammation with focal vasculitis consistent with GPA, and multiple bone cultures were positive for Pseudomonas aeruginosa. This patient was diagnosed concurrently with GPA and P. aeruginosa skull base osteomyelitis. He was started on a 6-week course of cefepime intravenously and oral prednisone, with the plan to initiate rituximab infusion 2 weeks after initiation of antibiotic therapy.

Original languageEnglish (US)
Article number220135
JournalBMJ Case Reports
Volume2017
DOIs
StatePublished - 2017

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Granulomatosis with Polyangiitis
Skull Base
Osteomyelitis
Pseudomonas aeruginosa
Posterior Cranial Fossa
Antineutrophil Cytoplasmic Antibodies
Headache Disorders
Vasculitis
Prednisone
Nose
Immunosuppression
Fatigue
Weight Loss
Rheumatoid Arthritis
Anti-Bacterial Agents
Inflammation
Biopsy
Bone and Bones
Antibodies
Therapeutics

Keywords

  • bone and joint infections
  • vasculitis

ASJC Scopus subject areas

  • Medicine(all)

Cite this

Severe destructive nasopharyngeal granulomatosis with polyangiitis with superimposed skull base Pseudomonas aeruginosa osteomyelitis. / Von Itzstein, Mitchell S.; Abeykoon, Jithma P.; Summerfield, Daniel D.; Whitaker, Jennifer A.

In: BMJ Case Reports, Vol. 2017, 220135, 2017.

Research output: Contribution to journalArticle

Von Itzstein, Mitchell S. ; Abeykoon, Jithma P. ; Summerfield, Daniel D. ; Whitaker, Jennifer A. / Severe destructive nasopharyngeal granulomatosis with polyangiitis with superimposed skull base Pseudomonas aeruginosa osteomyelitis. In: BMJ Case Reports. 2017 ; Vol. 2017.
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