Abstract
Objective To summarize the clinical features of autoimmune hypophysitis(AH)in Chinese. Methods We made a computer - based retrieval in CNKI and Wanfang with a search word " hypophysitis" and search time from January 1991 to December 2013. After the exclusion of repeated literatures and literatures without results of nuclear magnetic resonance examination on hypophysis,we included 48 pieces of literatures which included 166 AH patients. Relevant information was extracted from included literatures, incluing general data, clinical manifestations, laboratory examination, imageological examination and therapeutical methods. Results Among 166 patients,the male to female ratio was 1∶ 3. 88;the age of onset ranges from 18 to 71,with an average onset age of 35;15 patients had autoimmune hypophysitis onset after delivery,and 3 patients had onset in the later stage of pregnancy. The major clinical manifestations were polydipsia and polyuria(60. 8% ,101 /166 ), headache ( 54. 2% , 90 / 166 ), ocular symptoms ( 38. 6% , 64 / 166 ), fatigue ( 22. 9% , 38 / 166 ). The manifestations of pituitary function were dysfunction of the pituitary - adrenal axis(43. 5% ,67 / 154),dysfunction of pituitary - thyroid axis( 41. 6% ,64 / 154 ),dysfunction of pituitary - gonadal axis( 40. 2% ,62 / 154 ),elevation of PRL level (25. 0% ,33 / 132),decrease of growth hormone level(14. 7% ,5 / 34)and central diabetes insipidus(66. 4% ,95 / 143). Among all subjects,9 (5. 42% )patients had thyroid disease,8 (4. 8% )had diabetes mellitus,5 (3. 0% ) had sicca syndrome,2(1. 2% )had hemolytic anemia,2(1. 2% ) had autoimmune pancreatitis,2(1. 2% )had dacryoadenitis,1 (0. 6% )had reactive arthritis and 1(0. 6% )had systemic lupus erythematosus. Pituitary MRI showed that 123(74. 1% )had enlargement of pituitary gland,109(65. 7% ) had pituitary stalk enlargement,64(38. 6% ) had lift of optic chiasma by compression,48(28. 9% )showed lesion extended to cavernous sinus and 80(48. 2% )showed posterior pituitary high signal disappeared. Therapeutic methods were introduced by 148 subjects, among which 71 ( 48. 0% ) were treated with glucocorticoid,6(4. 0% ) were treated with glucocorticoid combined azathioprine and 73 (49. 3% ) were given operative treatment. Among 64 patients with secondary hypothyroidism,24(37. 5% )were given thyroxine replacement treatment;among 62 patients with secondary hypogonadism,2(3. 2% )were given hormone replacement therapy;among 95 patients with central diabetes insipidus,24(25. 3% )were given desmopressin replacement therapy. After 2 - day to 5 - month treatment,129 (87. 2% )patients saw their symptom relieved,and 19(14. 7% )of them relapsed and were treated again with glucocorticoid or glucocorticoid combined with azathioprine,and no patients relapsed later. Conclusion AH occurs more in women in latter half of gestation and postpartum woman,and it is rare in males. Based on characteristic clinical manifestations,pituitary MRI and effective glucocorticoid treatment,clinical diagnosis can be made. Glucocorticoid therapy should be taken as the priority in the treatment of AH. Transsphenoidal surgery should be chosen cautiously. Target hormone replacement therapy should be emphasized on patients with a lack of anterior pituitary hormone,in order to improve patients' quality of life.关键词
垂体疾病/自身免疫疾病/症状和体征/诊断/治疗Key words
Pituitary diseases/Autoimmune diseases/Symptoms and signs/Diagnosis/Therapy分类
医药卫生
