Eosinophilic sialodochitis: redefinition of ‘allergic parotitis’ and ‘sialodochitis fibrinosa’

Abstract

Sialodochitis fibrinosa and allergic parotitis have described rare patients with recurrent salivary gland swelling and mucus plugs, often with atopy. We have evaluated three patients with atopic disease, recurrent salivary gland swelling and an eosinophilic sialodochitis. Two had eosinophil-rich mucus plugs. 56 additional cases were identified in a medical literature database search, each defined by recurrent salivary gland swelling associated with eosinophil-rich mucus plugs or sialodochitis with periductal eosinophilic infiltration. The majority (78%) were reported from Japan. Females were predominantly affected (F:M=2.3) with a median age of 47 years at evaluation. The parotid and submandibular glands were involved, respectively, in 71% and 46%. Allergic symptoms were present in 66%, atopic disease in 63% of those with reported allergy testing, and blood eosinophilia in 71%. Contrast sialography and other imaging modalities documented ductal dilatation in 82%. Treatments included anti-allergic medications (58%), systemic glucocorticoids (25%), duct cannulation with irrigation, steroid injection, and/or duct dilatation (36%), and glandular resection (19%). We recommend the diagnosis “eosinophilic sialodochitis” be applied to patients who meet this case definition. The disease is a unique cause of chronic recurrent salivary gland swelling. Its likely allergic etiology may be amenable to current or future biologic therapies.

Sialodochitis fibrinosa: Salivary duct obstruction by eosinophil extracellular traps?

“…Histopathology of Wharton's duct and submandibular gland tissue from the patient revealed epithelial dislodgment and the presence of a dense periductal lymphoplasmacytic infiltrate, consistent with findings in previous reports (Baer et al., 2017; Flores et al., 2016). Immunohistochemical staining revealed the presence of lymphocytes, primarily CD4 + helper T cells (Figure ).…”

“…Sialodochitis fibrinosa (also known as Kussmaul disease) was originally described by Kussmaul in 1879. This condition involves recurrent parotid grand swelling and mucus plugs containing leucocytes and Charcot–Leyden crystals (CLCs; Baer, Okuhama, Eisele, Tversky, & Gniadek, 2017; Flores et al., 2016; Ray, Burgin, & Spector, 2015). Sialodochitis fibrinosa also includes recurrent salivary gland swelling with fibrinous eosinophil‐rich mucus plugs.…”

“…Sialodochitis fibrinosa also includes recurrent salivary gland swelling with fibrinous eosinophil‐rich mucus plugs. Although the disease is relatively rare, a recent review of Japanese and English literature revealed 59 previous cases (Baer et al., 2017). The authors of that review emphasised the major role of eosinophilic inflammation in sialodochitis fibrinosa and recommended redefinition of the disease as “eosinophilic sialodochitis.”…”

Eosinophilic Sialodochitis: A Type of Chronic Obstructive Sialadenitis Related to Allergy

“…In 1879, Kussmaul reported a disease characterized by recurrent parotid gland (PG) swelling and mucus plugs containing leukocytes and Charcot–Leyden crystals 4 . Since then, a similar recurrent major salivary gland swelling condition (Kussmaul disease) with mucus plugs and/or allergies has been termed allergic parotitis, 5 sialodochitis fibrinosa, 6,7 or eosinophilic sialodochitis (ES) 4 . Most reports indicate allergy as an etiology of ES and emphasize eosinophilia in both mucus plugs and peripheral blood, as well as elevated serum IgE.…”

“…Most reports indicate allergy as an etiology of ES and emphasize eosinophilia in both mucus plugs and peripheral blood, as well as elevated serum IgE. Histopathological studies show eosinophilic infiltration around the large salivary gland ducts 4,8–10 . Treatment for ES includes the abovementioned conventional options as well as anti‐anaphylactic treatment 4,7 …”