Glandular Manifestations of the Head and Neck in ANCA-Associated Vasculitides: Data from a National Cohort and Systematic Review.

Glandular involvement of the head and neck is an extremely rare feature in ANCA-associated vasculitis (AAV). The objective was to describe glandular involvement in AAV by analysing data from a national cohort and comparing the findings to those from a systematic literature review. A multicentric retrospective study was conducted through a survey promoted by the French Vasculitis Study Group and included patients aged ≥ 16 years who met the ACR/EULAR classification criteria for AAV and had lachrymal and/or salivary involvement. Demographic, clinical and biological findings were analysed and pooled with data from a MEDLINE review of the literature since inception until June 5, 2024. The study population included 20 patients with a median age of 52 years (IQR, 44.5-57.5 years). Granulomatosis with polyangiitis (n = 17) and PR3-ANCA (n = 10) were the most represented. Parotitis (n = 9) and dacryoadenitis (n = 8) were found to precede and/or were present at AAV onset and recurrences. All patients received glucocorticoids, usually in combination with immunosuppressants. The systematic review identified another 67 cases. Pooled analysis of all cases (n = 87) showed that salivary gland involvement was not only frequently associated with ENT features (OR = 4.01 (95% CI, 1.30-13.06; p < 0.01)), but also with PR3-ANCA positivity (OR = 3.34 (95% CI, 1.09-11.34; p = 0.02)). Lachrymal involvement was associated with concomitant ophthalmological signs (OR = 3.93 (95% CI, 1.31-12.14; p < 0.01)). Glandular involvement of the head and neck is a rare but identifiable manifestation of active AAV. Knowledge of this uncommon presentation refines clinical assessment for optimal management of patients with AAV.