Salivary gland involvement in ANCA-associated vasculitis

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.

We conducted a systematic literature review of articles reporting salivary gland involvement in ANCA-associated vasculitis from the inception dates until May 2, 2021.

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.

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.