Das Susanta Kumar, Ghosh Amritava, Banerjee Niloy, Khaski Sudarshan, Mukherjee Sabyasachi
Department of Medicine, RG Kar Medical College and Hospital, Kolkata.
J Indian Med Assoc. 2012 May;110(5):327, 329.
Polyglandular auto-immune syndromes are uncommon constellation of organ specific auto-immune diseases, characterised by the existence of two or more endocrinopathies. Polyglandular autoimmune type II syndrome also known as Schmidt's syndrome is more common, comprising Addison's disease, auto-immune thyroid disease, type 1 diabetes mellitus and/or hypogonadism, pernicious anaemia, coeliac disease, vitiligo, hypophysitis, etc. Here a case of a 56-year-old man is reported with a prior history of hypothyroidism who presented with adrenal crisis. Further laboratory investigations revealed primary auto-immune hypothyroidism, primary adrenal insufficiency, hypogonadism and he was diagnosed as a case of polyglandular auto-immune type II syndrome or Schmidt's syndrome. Early recognition of the syndrome and replacement therapy can be life saving, particularly when there is adrenal or thyroid insufficiency.
多腺体自身免疫综合征是一组罕见的器官特异性自身免疫性疾病,其特征是存在两种或更多种内分泌病。多腺体自身免疫II型综合征也称为施密特综合征,更为常见,包括艾迪生病、自身免疫性甲状腺疾病、1型糖尿病和/或性腺功能减退、恶性贫血、乳糜泻、白癜风、垂体炎等。本文报告一例56岁男性,既往有甲状腺功能减退病史,现出现肾上腺危象。进一步的实验室检查发现原发性自身免疫性甲状腺功能减退、原发性肾上腺皮质功能不全、性腺功能减退,他被诊断为多腺体自身免疫II型综合征或施密特综合征。早期识别该综合征并进行替代治疗可挽救生命,尤其是在存在肾上腺或甲状腺功能不全的情况下。