A case of anti-neutrophil cytoplasmic antibody-associated vasculitis masquerading as Sjögren syndrome

Published Date: 10th July 2023

Publication Authors: Pillay SS, Nune A, Raza H

Abstract
Anti-neutrophil cytoplasmic antibody (ANCA) -positive vasculitis is a small-vessel vasculitis that affects multiple body systems. Salivary gland involvement in ANCA-associated vasculitis is rare. When present, it mimics infection or malignancy, which might lead to misdiagnosis. In this report, we describe a 72-year-old man who presented with parotid and submandibular gland pain and swelling in addition to dry mouth and eyes. He had bilateral non-tender parotid gland lumps and no lymphadenopathies. Laboratory tests were positive for ANCA, hematuria, and proteinuria but negative for Anti-Ro and -La. He was treated with corticosteroids and cyclophosphamide for acute kidney injury. Unfortunately, the patient died a few months later. This case report sheds light on a rare manifestation of salivary gland involvement in ANCA-associated vasculitis that mimics Sjögren syndrome and the challenges associated with its diagnosis and treatment.

 

Pillay, SS.; Nune, A.; Raza, H.; Manzo, C.; Barman, B.;. (2023). A case of anti-neutrophil cytoplasmic antibody-associated vasculitis masquerading as Sjögren syndrome. International Journal of Rheumatic Diseases. 26(12), pp.2555-2558. [Online]. Available at: https://doi.org/10.1111/1756-185X.14823. [Accessed 26 February 2024]

 

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