INTRODUCTION
Granulomatosis with polyangiitis (GPA) is a rare form of small vessel
vasculitis characterized by multisystem necrotizing granulomatous
lesions with different organs involvements such as the central nervous
system, urinary tract, gastrointestinal tract, skin, and even eye (1-3).
The main pathological characteristic of GPA is the positivity of
autoantibodies against cytoplasmic components of neutrophil cells or
C-ANCA as well as proteinase-3 (PR3) over-activation (4). Due to the
multisystem involvement nature, the diagnosis of GPA is difficult.
Additionally, GPA may overlap with other clinicopathological conditions
such as infections, autoinflammatory disorders, connective tissue
diseases, and even neoplasms (5,6). In this regard, the involvement of
salivary glands is a very rare phenomenon. Herein, we described a case
of GPA with submandibular salivary gland involvement followed by
reviewing the literature on similar cases.