本文由小咖机器人翻译整理

期刊来源：Ann Intern Med

原文链接：https://doi.org/10.7326/M23-2024

摘要内容如下：

背景

糖皮质激素抑制炎症。库欣病（CD）缓解后可发生自身免疫性疾病。然而，在这种情况下，自身免疫性疾病的发展并没有得到很好的描述。

客观

确定1）与无功能垂体腺瘤（NFPA）患者相比，CD患者手术缓解后自身免疫性疾病的发生率；2）CD患者缓解后自身免疫性疾病发生的临床表现和危险因素。

设计

回顾性配对队列分析。

设置

学术医学中心/垂体中心。

病人

手术缓解和手术治疗NFPA的CD患者。

测量

术后3年新发自身免疫性疾病的累积发病率皮质醇增多症的评估包括深夜唾液皮质醇水平、24小时尿游离皮质醇（UFC）比率（UFC值除以测定的正常范围上限）和地塞米松抑制试验。

结果

术后3年，CD患者新发自身免疫性疾病的累积发病率（10.4%[95%CI，5.7%-15.1%]）高于NFPA患者（1.6%[95%CI，0%-4.6%]）（风险比，7.80[95%CI，2.88-21.10]）。与NFPAs患者相比，CD患者术后肾上腺功能不全的发生率较高（93.8%vs.16.5%），术后血清皮质醇水平的最低值较低（63.8 vs.282.3 nmol/L）。与无自身免疫性疾病的CD患者相比，发生自身免疫性疾病的患者术前24小时UFC比率较低（2.7 vs.6.3），且自身免疫性疾病家族史的患病率较高（41.2%vs.20.9%）。

局限性

自身免疫性疾病患者的小样本限制了独立危险因素的识别。

结论

获得手术缓解的CD患者比年龄和性别匹配的NFPA患者有更高的自身免疫性疾病发病率。自身免疫性疾病家族史是潜在的危险因素。肾上腺功能不全可能是诱因。

主要资金来源

Recordati罕见病公司

英文原文如下：

Abstracts

BACKGROUND  Glucocorticoids suppress inflammation. Autoimmune disease may occur after remission of Cushing's disease (CD). However, the development of autoimmune disease in this context is not well described.

OBJECTIVE  To determine 1) the incidence of autoimmune disease in patients with CD after surgical remission compared with patients with nonfunctioning pituitary adenomas (NFPAs) and 2) the clinical presentation of and risk factors for development of autoimmune disease in CD after remission.

DESIGN  Retrospective matched cohort analysis.

SETTING  Academic medical center/pituitary center.

PATIENTS  Patients with CD with surgical remission and surgically treated NFPA.

MEASUREMENTS  Cumulative incidence of new-onset autoimmune disease at 3 years after surgery. Assessment for hypercortisolemia included late-night salivary cortisol levels, 24-hour urine free cortisol (UFC) ratio (UFC value divided by the upper limit of the normal range for the assay), and dexamethasone suppression tests.

RESULTS  Cumulative incidence of new-onset autoimmune disease at 3 years after surgery was higher in patients with CD (10.4% [95% CI, 5.7% to 15.1%]) than in those with NFPAs (1.6% [CI, 0% to 4.6%]) (hazard ratio, 7.80 [CI, 2.88 to 21.10]). Patients with CD showed higher prevalence of postoperative adrenal insufficiency (93.8% vs. 16.5%) and lower postoperative nadir serum cortisol levels (63.8 vs. 282.3 nmol/L) than patients with NFPAs. Compared with patients with CD without autoimmune disease, those who developed autoimmune disease had a lower preoperative 24-hour UFC ratio (2.7 vs. 6.3) and a higher prevalence of family history of autoimmune disease (41.2% vs. 20.9%).

LIMITATION  The small sample of patients with autoimmune disease limited identification of independent risk factors.

CONCLUSION  Patients achieving surgical remission of CD have higher incidence of autoimmune disease than age- and sex-matched patients with NFPAs. Family history of autoimmune disease is a potential risk factor. Adrenal insufficiency may be a trigger.

PRIMARY FUNDING SOURCE  Recordati Rare Diseases Inc.

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