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.%目的：总结我国自身免疫性垂体炎（AH）患者临床特征。方法计算机检索中国知网、万方数据库，检索词为“垂体炎”，时间限定为1991年1月—2013年12月，排除重复文献和无垂体磁共振成像检查结果的文献，共纳入48篇文献，报道 AH 患者166例。提取纳入文献相关信息，包括一般资料、临床表现、实验室检查、影像学检查及治疗方法。结果166例患者男∶女比例为1∶3.88；发病年龄18~71岁，平均发病年龄35岁；产后发病15例，妊娠后期发病3例。患者主要临床表现为多饮多尿（60.8％，101/166）、头痛（54.2％，90/166）、眼部症状（38.6％，64/166）、乏力（22.9％，38/166）等。垂体功能表现为垂体-肾上腺轴功能减退占43.5％（67/154），垂体-甲状腺轴功能减退占41.6％（64/154），垂体-性腺轴功能减退占40.2％（62/154），催乳素水平升高占25.0％（33/132），生长激素水平降低占14.7％（5/34），中枢性尿崩症占66.4％（95/143）。患者合并甲状腺疾病9例（5.42％），合并糖尿病8例（4.8％），合并干燥综合征5例（3.0％），合并溶血性贫血、自身免疫性胰腺炎、泪腺炎各2例（1.2％），合并反应性关节炎、系统性红斑狼疮各1例（0.6％）。行垂体磁共振成像检查显示，垂体增大123例（74.1％），垂体柄增粗109例（65.7％），视交叉受压上抬64例（38.6％），病变延伸至海绵窦48例（28.9％），神经垂体高信号消失80例（48.2％）。148例患者介绍了治疗方法，糖皮质激素治疗71例（48.0％），糖皮质激素联合硫唑嘌呤6例（4.0％），手术治疗73例（49.3％）。64例继发性甲状腺功能减退症患者中予甲状腺激素替代治疗24例（37.5％），62例继发性性功能减退症患者中予性激素替代治疗2例（3.2％），95例中枢性尿崩症患者中予去氨加压素替代治疗24例（25.3％）。经治疗2 d ~5个月，129例（87.2％）患者症状均明显好转，其中19例（14.7％）复发，经糖皮质激素或糖皮质激素联合硫唑嘌呤治疗后好转，未再复发。结论 AH 好发于妊娠后期及产后妇女，男性少见。在特征性的临床表现基础上结合垂体影像学检查，且糖皮质激素治疗有效，可做出临床诊断。AH 应首选糖皮质激素治疗，而经蝶手术治疗应持谨慎态度，同时应重视存在腺垂体激素缺乏患者补充靶激素治疗，以改善患者生活质量。
展开▼
