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患有和未患有艾迪生病的卵巢早衰患者体内针对类固醇生成酶的自身抗体。

Autoantibodies to steroidogenic enzymes in patients with premature ovarian failure with and without Addison's disease.

作者信息

Dal Pra C, Chen S, Furmaniak J, Smith B Rees, Pedini B, Moscon A, Zanchetta R, Betterle C

机构信息

Chair of Clinical Immunology and Allergy, Division of Endocrinology, Department of Medical and Surgical Sciences, University of Padua, Via Ospedale 105, Padua, Italy.

出版信息

Eur J Endocrinol. 2003 May;148(5):565-70. doi: 10.1530/eje.0.1480565.

Abstract

DESIGN

Adrenal cortex autoantibodies (ACA), steroid-producing cell autoantibodies (StCA) and autoantibodies (Abs) to steroidogenic enzymes in three groups of patients with premature ovarian failure (POF), 15 with autoimmune Addison's disease (AD), 26 with non-adrenal autoimmune diseases and 31 with isolated POF, have been assessed.

METHODS

ACA and StCA were measured using an immunofluorescence technique. Abs to 21-hydroxylase (21-OH), to 17alpha-hydroxylase (17alpha-OH) and to cytochrome P450 side-chain cleavage (P450scc) were measured using an immunoprecipitation assay.

RESULTS

Seventy-three percent of patients with POF and AD were positive for StCA, 93% for 17alpha-OH and/or P450scc Abs, 93% for ACA and 100% for 21-OH Abs. Among patients with POF and non-adrenal autoimmune diseases, 8% were positive for StCA, 12% for 17alpha-OH and/or P450scc Abs, and 8% and 12% for ACA and 21-OH Abs respectively. StCA, 17alpha-OH and/or P450scc Abs were all found in 10% of patients with isolated POF, and 13% had ACA and 21-OH Abs. All StCA-, 17alpha-OH- and/or P450scc Abs-positive patients were also positive for ACA and 21-OH Abs. Two patients with isolated POF who were ACA and 21-OH Ab positive developed AD 3 and 5 Years after the onset of POF.

CONCLUSION

This study has shown that, when POF is associated with AD, StCA, 17alpha-OH and/or P450scc Abs are present in the majority of patients, while in the other two groups these Abs are detectable in a much lower proportion of patients. Measurement of ACA/21-OH Abs in some patients with POF may be important in identifying patients at risk of developing overt AD.

摘要

设计

对三组卵巢早衰(POF)患者、15例自身免疫性 Addison 病(AD)患者、26例非肾上腺自身免疫性疾病患者以及31例孤立性POF患者的肾上腺皮质自身抗体(ACA)、类固醇生成细胞自身抗体(StCA)和类固醇生成酶自身抗体(Abs)进行了评估。

方法

采用免疫荧光技术检测ACA和StCA。采用免疫沉淀法检测21-羟化酶(21-OH)、17α-羟化酶(17α-OH)和细胞色素P450侧链裂解酶(P450scc)的抗体。

结果

POF合并AD患者中,73%的患者StCA呈阳性,93%的患者17α-OH和/或P450scc抗体呈阳性,93%的患者ACA呈阳性,100%的患者21-OH抗体呈阳性。在POF合并非肾上腺自身免疫性疾病的患者中,8%的患者StCA呈阳性,12%的患者17α-OH和/或P450scc抗体呈阳性,ACA和21-OH抗体呈阳性的患者分别为8%和12%。在孤立性POF患者中,10%的患者检测到StCA、17α-OH和/或P450scc抗体,13%的患者ACA和21-OH抗体呈阳性。所有StCA、17α-OH和/或P450scc抗体阳性的患者ACA和21-OH抗体也呈阳性。两名孤立性POF患者,ACA和21-OH抗体呈阳性,在POF发病3年和5年后发生了AD。

结论

本研究表明,当POF与AD相关时,大多数患者存在StCA、17α-OH和/或P450scc抗体,而在其他两组患者中,这些抗体的检出率要低得多。对一些POF患者检测ACA/21-OH抗体可能对识别有发生显性AD风险的患者很重要。

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