Korean Journal of Head & Neck Oncology (대한두경부종양학회지)

The Korean Society for Head and Neck Oncology (대한두경부종양학회)
권호 표지
DOI QR코드

DOI QR Code

A Case of Granulomatosis with Polyangiitis with Submandibular Abscess as Primary Manifestation

악하부 농양으로 발병한 육아종증 다발 혈관염 1예

  • Hyun Woong Jun (Department of Otolaryngology-Head and Neck Surgery, College of Medicine, Hanyang University) ;
  • Seong Man Hong (Department of Otolaryngology-Head and Neck Surgery, College of Medicine, Hanyang University) ;
  • Jin Hyeok Jeong (Department of Otolaryngology-Head and Neck Surgery, College of Medicine, Hanyang University) ;
  • Yong-Bae Ji (Department of Otolaryngology-Head and Neck Surgery, College of Medicine, Hanyang University)
  • 전현웅 (한양대학교 의과대학 이비인후-두경부외과학교실) ;
  • 홍성만 (한양대학교 의과대학 이비인후-두경부외과학교실) ;
  • 정진혁 (한양대학교 의과대학 이비인후-두경부외과학교실) ;
  • 지용배 (한양대학교 의과대학 이비인후-두경부외과학교실)
  • Received : 2022.10.12
  • Accepted : 2022.12.12
  • Published : 2023.05.30
Download PDF
⟨ Previous Next ⟩

Abstract

Granulomatosis with polyangiitis (GPA), formerly known as Wegener's granulomatosis, is a rare immunological small vessel disease which usually affects respiratory tract and kidneys. However, salivary gland involvement in GPA is rare as a primary manifestation. We report a case of GPA with the primary presentation of submandibular gland involvement. A 48-year-old female patient presented submandibular swelling with a skin defect that lasted for 1 month. Although the biopsy result was chronic inflammation, the skin defect did not heal for a month. Further imaging study revealed multiple lung and renal masses. More clinical manifestations such as gingivitis, ischemic change of finger joint and nasal tip skin, and positive c-ANCA test was presented. Additional biopsy was made at the submandibular gland, lung, and finger skin. The patient was finally diagnosed with GPA and treated with steroid pulse therapy and cyclophosphamide. The patient showed improvement of prior clinical symptoms.

Keywords

Acknowledgement

This research was supported by the Basic Science Research Program through the National Research Foundation of Korea (NRF) funded by the Ministry of Education (2021R1I1A4A01051258).

References

  1. Park KT, Kong IG, Han DH, Kim DW, Kim SH, Rhee CS, et al. Clinical Experiences of Diagnosis and Treatment for Wegener's Granulomatosis. Korean J Otorhinolaryngol-Head Neck Surg. 2008;51:1109-1118.
  2. Barrett AW. Wegener's granulomatosis of the major salivary glands. J Oral Pathol Med. 2012;41:721-727. https://doi.org/10.1111/j.1600-0714.2012.01141.x
  3. Greco A, Marinelli C, Fusconi M, Macri GF, Gallo A, De Virgilio A, et al. Clinic manifestations in granulomatosis with polyangiitis. Int J Immunopathol Pharmacol. 2016;29:151-159. https://doi.org/10.1177/0394632015617063
  4. Lynch JP, 3rd, Derhovanessian A, Tazelaar H, Belperio JA. Granulomatosis with Polyangiitis (Wegener's Granulomatosis): Evolving Concepts in Treatment. Semin Respir Crit Care Med. 2018;39:434-458. https://doi.org/10.1055/s-0038-1660874
  5. Cleary JO, Sivarasan N, Burd C, Connor SEJ. Head and neck manifestations of granulomatosis with polyangiitis. Br J Radiol. 2021;94:20200914.
  6. Anderson G, Coles ET, Crane M, Douglas AC, Gibbs AR, Geddes DM, et al. Wegener's granuloma. A series of 265 British cases seen between 1975 and 1985. A report by a sub-committee of the British Thoracic Society Research Committee. Q J Med. 1992;83:427-438.
  7. Tsuzuki K, Fukazawa K, Takebayashi H, Hashimoto K, Sakagami M. Difficulty of diagnosing Wegener's granulomatosis in the head and neck region. Auris Nasus Larynx. 2009;36:64-70. https://doi.org/10.1016/j.anl.2008.02.003
  8. Nahlieli O. Wegener's granulomatosis and the salivary glands. Isr Med Assoc J. 2013;15:178-179.
  9. Vanhauwaert BG, Roskams TA, Vanneste SB, Knockaert DC. Salivary gland involvement as initial presentation of Wegener's disease. Postgrad Med J. 1993;69:643-645. https://doi.org/10.1136/pgmj.69.814.643
  10. Geyer M, Kulamarva G, Davis A. Wegener's Granulomatosis presenting with an abscess in the parotid gland: a case report. J Med Case Rep. 2009;3:19.
  11. Pagnoux C, Guillevin L. Treatment of granulomatosis with polyangiitis (Wegener's). Expert Rev Clin Immunol. 2015;11:339-348. https://doi.org/10.1586/1744666X.2015.1008455
  12. Abdelghany MF, Khalaf MGA. Submandibular salivary gland involvement in granulomatosis with polyangiitis. Egyptian Journal of Bronchology. 2020;13:781-785. https://doi.org/10.4103/ejb.ejb_15_19