Primary Hyper-IgE-Related Salivary Gland Disease: A New Disease Entity.

OBJECTIVES: To clarify the clinicopathological characteristics of primary hyper-IgE-related salivary gland disease (PHIESD), which is a newly proposed entity. METHODS: Fifteen consecutive patients pathologically diagnosed with chronic sialadenitis were enrolled, and their clinicopathological features were comprehensively analyzed. INCLUSION CRITERIA: (1) multiple salivary gland enlargement; (2) elevated serum IgE and/or IgE-positive cell infiltration in salivary gland tissues; (3) histology-confirmed lymphoplasmacytic infiltration; (4) exclusion of other known diseases. RESULTS: The male-to-female ratio was 5:10. The median age was 21 (range, 3-63) years. The average number of affected glands was 3.7 +/- 1.4. Submandibular, parotid, and sublingual glands were involved in 15, 8, and 2 patients, respectively. Comorbid diseases included allergic diseases in seven patients and autoimmune diseases in two. Elevated serum IgE (median 175 kU/L) was seen in all patients. Serum IgG4 was slightly elevated in three patients. Histologically, most patients had mild lesions, including mild lymphocyte infiltration (60%) and focal fibrosis (66.7%). Lymphoid follicular formation (53.3%), moderate to severe lymphocytic inflammation (40%) and severe fibrosis (33.3%) were also observed. Immunohistochemically, IgE-positive cells infiltrated mainly around the ducts, with scattered infiltration of IgG4-positive, mast, and interleukin-4 positive cells. During follow-up (median, 46 months) of ten patients without intervention and two with immunosuppressive therapy, no significant changes in gland size or serum IgE level were noted. CONCLUSIONS: PHIESD manifests as homogeneous enlargement of multiple salivary glands and elevated serum IgE. Histopathology further verifies the diagnosis. It might be associated with anaphylaxis or autoimmune dysfunction. Conservative treatment is suggested. LEVEL OF EVIDENCE: 4 Laryngoscope, 132:2132-2138, 2022.