Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
. 2024 Dec 9;11(12):004992.
doi: 10.12890/2024_004992. eCollection 2024.

A Rare Case of Limited Granulomatosis with Polyangiitis Presenting as Bilateral Parotitis

Vandana Bandari  1 , Sandra Apenteng  2 , Aaradhana Kaul  2
Affiliations

A Rare Case of Limited Granulomatosis with Polyangiitis Presenting as Bilateral Parotitis

Vandana Bandari et al. Eur J Case Rep Intern Med. .

Abstract

Background: Granulomatosis with polyangiitis (GPA) is a rare autoimmune vasculitis affecting small and medium-sized vessels, commonly involving the respiratory tract and kidneys. Salivary gland involvement, particularly bilateral parotitis, is an uncommon presentation of GPA.

Case report: We report the case of a 38-year-old Asian male who presented with left ear pain and parotid swelling after a water park visit. Initially treated with antibiotics for suspected otitis externa, his symptoms worsened, leading to bilateral parotitis, facial palsy, and otomastoiditis. Imaging revealed parotid abscesses and lab results showed elevated antineutrophil cytoplasmic antibodies (ANCA), specifically cytoplasmic ANCA directed against proteinase 3, confirming the diagnosis of GPA. Cultures and autoimmune workups for other causes, such as Sjogren's syndrome and immunoglobulin G4-related disease, were negative. Treatment with high-dose corticosteroids and methotrexate resulted in significant clinical improvement.

Conclusion: This case highlights the importance of considering GPA in atypical presentations such as parotitis, even in the absence of renal involvement. Early diagnosis and appropriate immunosuppressive therapy are critical to prevent further complications in multisystem involvement.

Learning points: This case highlights the importance of recognizing atypical manifestations of granulomatosis with polyangiitis (GPA), such as bilateral parotitis, which aids in diagnosing and treating rare presentations more effectively.It emphasizes the need to expand differential diagnoses of salivary gland involvement, guiding the distinction of GPA from other similar conditions when antibiotics fail.By highlighting a rare presentation of GPA, this case improves diagnostic understanding in autoimmune diseases, enabling earlier interventions and leading to better patient outcomes.

Keywords: Limited granulomatosis with polyangiitis; bilateral parotitis; parotitis.

PubMed Disclaimer

Conflict of interest statement

Conflicts of Interests: The Authors declare that there are no competing interests.

Figures

Figure 1
Figure 1
Swelling and tenderness of the preauricular area.
Figure 2
Figure 2
Magnetic resonance imaging of A) T2, B) axial pre-contrast and C) axial post-contrast soft tissue.
See this image and copyright information in PMC

Similar articles

See all similar articles

References

    1. Chandwar K, Kishor K, Mukherjee S, Dhakad U. Salivary gland involvement in granulomatosis with polyangiitis: case report and review of literature. Indian J Rheumatol. 2022;18:86–93.
    1. Panupattanapong S, Stwalley DL, White AJ, Olsen MA, French AR, Hartman ME. Epidemiology and outcomes of granulomatosis with Polyangiitis in pediatric and working-age adult populations In the United States: analysis of a large National Claims Database. Arthritis Rheumatol. 2018;70:2067–2076. - PMC - PubMed
    1. Kyurkchiev D, Yoneva T, Yordanova A, Kurteva E, Vasilev G, Zdravkova Y, et al. Alterations of serum levels of plasminogen, TNF-α, and IDO in granulomatosis with polyangiitis patients. Vascular. 2021;29:874–882. - PubMed
    1. Chen M, Kallenberg CG. New advances in the pathogenesis of ANCA-associated vasculitides. Clin Exp Rheumatol. 2009;27:S108–114. - PubMed
    1. Dias B, Soares D, Sampaio P, Santiago M. Granulomatosis with polyangiitis presenting as a parotid gland abscess. Case Rep Med. 2015;2015:865108. - PMC - PubMed

LinkOut - more resources