Clinical Analysis And Current Research Status Of IgG4-related Sialadenitis

Objective:By analysing clinical characteristics, serology, pathologic features, treatment and prognosis of IgG4-related sialadenitis, to improve understanding of this disease.Methods:Retrospective analysis of clinical features, serology, pathologic changes, treatment and prognosis in6cases of IgG4-related sialadenitis from The Second Affiliated Hospital of Zhejiang University Medical College in the past5years (Jan.2009to Dec.2014) with review of the relevant literatures.Results:The6cases go for a doctor all because of salivary glands painless swelling or mass without a remarkable reason;5male cases and1female case with a male-to-female ratio of5to1; Age is from50years to76years, the mean age is62years, median age is64years. The involvement sites include bilateral submandibular gland and lacrimal gland (3cases), bilateral submandibular gland (2cases), right parotid and submandibular region (1case). Serum IgG4concentration is significantly elevated (higher than1.35g/L) in all tesed cases, from6.89g/L to34.60g/L. High IgG and IgE level, low complement can be observed in some cases. The autoimmune antibodies are negative in all tested5cases. Ultrasound examation shows salivary gland enlargement or low echogenic areas in gland. MRI indicates inflammatory changes in involved gland. In histopathology, salivary glands show abundant lymphoplasmacytic cells infiltration with fibrosis, high numbers of IgG4-positive plasma cells can be observed on immunostaining, usually IgG4-positive plasma cells are more than50per high-power field as well as with IgG4/IgG ratio at least40%. Some cases are correctly diagnosed after salivary gland resection by pathology, then they go to Rheumatology and Immunology Department for furture treatment. This disease responds well to glucocorticoid therapy alone or combined with immunosuppression, swell remission and decrease of serum IgG4can be observed, long-time effect is still under follow-up. Liver involment is found in1case, other IgG4related disease is not found in rest cases.Conclusions:IgG4-related sialadenitis is predominantly elders. Salivary glands painless swelling, elevated serum IgG4level and abundant IgG4-positive plasma cells infiltration with fibrosis are the most significant features. When IgG4-related sialadenitis is suspected, serology test and pathological biopsy is necessary for diagnose, and an early diagnose and treatment improves prognosis. Glucocorticoid is the first-line drug, with a good effect of swell remission and serum IgG4decrease, immunosuppression is also used for more effective therapy or less relapse.