艾迪生病和自身免疫性多内分泌腺病Ⅲ型中针对重组人甾体生成酶21-羟化酶、侧链裂解酶和17α-羟化酶的自身抗体。

Autoantibodies against recombinant human steroidogenic enzymes 21-hydroxylase, side-chain cleavage and 17alpha-hydroxylase in Addison's disease and autoimmune polyendocrine syndrome type III.

作者信息

de Carmo Silva R, Kater C E, Dib S A, Laureti S, Forini F, Cosentino A, Falorni A

机构信息

Division of Endocrinology, Department of Medicine, Universidade Federal de Sâo Paulo, Escola Paulista de Medicina, Sâo Paulo, SP, Brazil.

出版信息

Eur J Endocrinol. 2000 Feb;142(2):187-94. doi: 10.1530/eje.0.1420187.

DOI:10.1530/eje.0.1420187

PMID:10664529

Abstract

OBJECTIVE

To evaluate the frequency of autoantibodies (Ab) against 21 hydroxylase (21OH), side-chain cleavage (SCC) and 17alpha-hydroxylase (17OH), in Addison's disease (AD) and autoimmune polyendocrine syndrome type III (APSIII).

DESIGN AND METHODS

We used radiobinding assays and in vitro translated recombinant human (35)S-21OH, (35)S-SCC or (35)S-17OH and studied serum samples from 29 AD (18 idiopathic, 11 granulomatous) and 18 APSIII (autoimmune thyroid disease plus type 1 diabetes mellitus, without AD) patients. Results were compared with those of adrenocortical autoantibodies obtained with indirect immunofluorescence (ACA-IIF).

RESULTS

ACA-IIF were detected in 15/18 (83%) idiopathic and in 1/11 (9%) granulomatous AD subjects. 21OHAb were found in 14/18 (78%) idiopathic and in the same (9%) granulomatous AD subject. A significant positive correlation was shown between ACA-IIF and 21OHAb levels (r(2)=0.56, P<0.02). The concordance rate between the two assays was 83% (24/29) in AD patients. SCCAb were found in 5/18 (28%) idiopathic (4 of whom were also positive for 21OHAb) and in the same (9%) granulomatous AD subject. 17OHAb were found in only 2/18 (11%) idiopathic and none of the granulomatous AD patients. Two APSIII patients were positive for ACA-IIF, but only one was positive for 21OHAb and SCCAb. 17OHAb were found in another two APSIII patients.

CONCLUSIONS

Measurement of 21OHAb should be the first step in immune assessment of patients with AD and individuals at risk for adrenal autoimmunity, in addition to ACA-IIF. Due to their low prevalence in AD, measurement of SCCAb and 17OHAb should be indicated only for 21OHAb negative patients and/or for those with premature ovarian failure, regardless of ACA-IIF results.

摘要

目的

评估自身免疫性 Addison 病（AD）和自身免疫性多内分泌综合征 III 型（APSIII）患者中抗 21 羟化酶（21OH）、侧链裂解酶（SCC）和 17α - 羟化酶（17OH）自身抗体（Ab）的出现频率。

设计与方法

我们采用放射结合试验以及体外翻译的重组人（35）S - 21OH、（35）S - SCC 或（35）S - 17OH，研究了 29 例 AD 患者（18 例特发性、11 例肉芽肿性）和 18 例 APSIII 患者（自身免疫性甲状腺疾病加 1 型糖尿病，无 AD）的血清样本。将结果与通过间接免疫荧光法（ACA - IIF）获得的肾上腺皮质自身抗体结果进行比较。

结果

15/18（83%）的特发性 AD 患者和 1/11（9%）的肉芽肿性 AD 患者检测到 ACA - IIF。14/18（78%）的特发性 AD 患者和相同的 1/11（9%）肉芽肿性 AD 患者检测到 21OHAb。ACA - IIF 与 21OHAb 水平之间显示出显著正相关（r² = 0.56，P < 0.02）。AD 患者中两种检测方法的一致性率为 83%（24/29）。5/18（28%）的特发性 AD 患者（其中 4 例 21OHAb 也呈阳性）和相同的 1/11（9%）肉芽肿性 AD 患者检测到 SCCAb。仅 2/18（11%）的特发性 AD 患者检测到 17OHAb，肉芽肿性 AD 患者均未检测到。2 例 APSIII 患者 ACA - IIF 呈阳性，但仅 1 例 21OHAb 和 SCCAb 呈阳性。另外 2 例 APSIII 患者检测到 17OHAb。