关键词: IgG4相关性疾病, IgG4相关性涎腺炎, 慢性硬化性涎腺炎

Abstract:

Chronic sclerosing sialadenitis(CSS) is a kind of chronic sialadenitis, frequently involved unilateral or bilateral submandibular gland, manifested as tough pain or painless mass, and it is multiple in adult male. Its pathogenesis may be related to genetic predisposition and abnormal immune response. Laboratory examination showed the increased IgG4. CSS specific pathological features are proliferation of cellulous fibrous tissue, formation of lymphoid follicles with obvious irregular shape of the germinal centers, and a large number of IgG4 positive plasma cells infiltration is its main diagnosis basis. Glucocorticoid is considered to be the most effective drugs against the IgG4 related disease, but the recurrence rate of the treatment is high. The disease has more than a hundred years of history, but its pathogenesis, diagnosis standard and effective treatment remains uncertain, we believe that with the more widely and deeply research of the disease, the above problems will be solved gradually.

Key words: IgG4 related disease, IgG4 related sialadenitis, chronic sclerosing sialadenitis