Objective: Salivary gland involvement in anti-neutrophil cytoplasmic autoantibody (ANCA)-associated vasculitis is rare, but can lead to the misdiagnosis of other diseases. The objective of this study was to clarify the characteristics of patients with salivary gland involvement. Methods: We conducted a systematic literature review of articles reporting salivary gland involvement in ANCA-associated vasculitis from the inception dates until May 2, 2021. Results: We identified 58 patients with salivary gland involvement. The mean age was 52 years, and men were predominantly affected (59%). Half of the patients presented with fever. Swelling of the salivary gland was the initial manifestation in 88% of the patients, unilaterally affected in 53%, and painful in 47%. The affected salivary glands were as follows: parotid gland alone (53%), submandibular gland alone (33%), and both parotid and submandibular glands (14%). Additionally, two patients had sublingual gland involvement. The most frequent clinical diagnosis was granulomatosis with polyangiitis (83%), followed by eosinophilic granulomatosis with polyangiitis (17%), while no patient was diagnosed with microscopic polyangiitis. PR3-ANCA positivity (72%) was predominant to MPO-ANCA positivity (6%), and ANCA was negative in 22% of the patients. Among 37 ANCA-positive patients, 6 patients (16%) were initially ANCA-negative, but subsequently became positive during the clinical course. The serum C-reactive protein levels were elevated in all the examined patients. On contrast-enhanced computed tomography, a finding suggestive of necrosis, which was heterogeneous enhancement with low-density areas, was found in 33% of the patients. Vasculitis, granulomatous inflammation, necrosis, or the presence of multinucleated giant cells was found in 83% of the biopsy samples of the affected salivary gland. Glucocorticoids with or without other immunosuppressive agents, such as cyclophosphamide were effective in most patients, but twelve patients (21%) experienced a relapse of the disease and nine patients (16%) died during the clinical course. Conclusion: Salivary gland involvement can be an initial manifestation of ANCA-associated vasculitis. The recognition of this unusual atypical presentation is important for the early and accurate diagnosis and treatment.
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