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

Abstract

AbstractAnti‐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.