多腺体自身免疫综合征

Polyglandular Autoimmune Syndrome

多腺体自身免疫综合征为以一种内分泌腺功能障碍为主,而同时并有其它内分泌或非分泌器官自身免疫疾患的综合征。临床分为3型:PAS I型以甲状旁腺功能低下为主;Ⅱ型以阿迪森氏病为主,Ⅲ型以自身免疫性甲状腺炎为主病。PAS合并非内分泌器官病变目增。淋巴细胞浸润为其主要病理变化。本文报告PASⅢ型10例。2病并发者5例;3病并发者5例。经强地松及/或甲状腺片治疗后均缓解。

Polyglandular autoimmune syndrome (PAS) is characterized by the dysfunction of the endocrine gland associated with other organ autoimmune disorders. There are three types: PAS type I: chief disorder-hypoparathyroidism; type Ⅱ: chief disorder-adrenal insufficiency; type Ⅲ: autoimmune thyroiditis without Addison's disease but with one or more other autoimmune diseasesRecently, the incidence of autoiommune disease involving nonendocrine organs has increased Lymphocytic infiltration is a prominent pathologic feature of the organ involved.In this article, the authors report on ten cases of PAS-type Ⅲ. Five of the patients were associated with two autoimmune diseases and five cases were associated with three manifestations. Thyroid hormone and low dose prednisone treatment resulted in satisfactory improvement.