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Rare disease
CASE REPORT
Severe destructive nasopharyngeal granulomatosis with polyangiitis with superimposed skull base Pseudomonas aeruginosa osteomyelitis
  1. Mitchell S von Itzstein1,
  2. Jithma P Abeykoon2,
  3. Daniel D Summerfield3,
  4. Jennifer A Whitaker4
  1. 1School of Medicine, Griffith University, Gold Coast, Australia
  2. 2Department of Internal Medicine, Mayo Clinic, Rochester, Minnesota, USA
  3. 3Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, Minnesota, USA
  4. 4Division of Infectious Diseases, Mayo Clinic, Rochester, Minnesota, USA
  1. Correspondence to Dr Jennifer A Whitaker, whitaker.jennifer{at}mayo.edu

Summary

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.

  • bone and joint infections
  • vasculitis

This is an Open Access article distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/4.0/

https://doi.org/10.1136/bcr-2017-220135

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    Footnotes

    • Contributors MSvI, JPA, DDS and JAW contributed to the conception and design of this case report, interpretation of data, critical revision and final approval of the manuscript. All authors agree to be accountable for the article and to ensure that all questions regarding the accuracy or integrity of the article are investigated and resolved.

    • Competing interests None declared.

    • Patient consent Obtained from guardian.

    • Provenance and peer review Not commissioned; externally peer reviewed.