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一名年轻女性中甲状腺炎、艾迪生病、卵巢功能衰竭和乳糜泻的罕见关联。

Unusual association of thyroiditis, Addison's disease, ovarian failure and celiac disease in a young woman.

作者信息

Valentino R, Savastano S, Tommaselli A P, Dorato M, Scarpitta M T, Gigante M, Lombardi G, Troncone R

机构信息

Centro di Endocrinologia e Oncologia Sperimentale del CNR, Dipartimento di Biologia e Patologia Cellulare e Molecolare L. Califano, Italy.

出版信息

J Endocrinol Invest. 1999 May;22(5):390-4. doi: 10.1007/BF03343578.

DOI:10.1007/BF03343578
PMID:10401714
Abstract

The coexistence of autoimmune endocrine diseases, particularly autoimmune thyroid disease and celiac disease (CD), has recently been reported. We here present a 23-year-old woman with a diagnosis of hypothyroidism due to Hashimoto's thyroiditis, autoimmune Addison's disease, and kariotypically normal spontaneous premature ovarian failure. Considering the close association between autoimmune diseases and CD, we decided to search for IgA anti-endomysium antibodies (EmA) in the serum. The positivity of EmA and the presence of total villous atrophy at jejunal biopsy allowed the diagnosis of CD. On a gluten-free diet the patient showed a marked clinical improvement accompanied, over a 3-month period, by a progressive decrease in the need for thyroid and adrenal replacement therapies. After 6 months, serum EmA became negative and after 12 months a new jejunal biopsy showed complete mucosal recovery. After 18 months on gluten-free diet, the anti-thyroid antibodies titre decreased significantly, and we could discontinue thyroid substitutive therapy. This case emphasizes the association between autoimmune polyglandular disease and CD; the precocious identification of these cases is clinically relevant not only for the high risk of complications (e.g. lymphoma) inherent to untreated CD, but also because CD is one of the causes for the failure of substitute hormonal therapy in patients with autoimmune thyroid disease.

摘要

自身免疫性内分泌疾病,尤其是自身免疫性甲状腺疾病和乳糜泻(CD)的共存情况,近来已有报道。我们在此报告一名23岁女性,诊断为桥本甲状腺炎所致甲状腺功能减退、自身免疫性艾迪生病,以及核型正常的自发性卵巢早衰。鉴于自身免疫性疾病与CD之间的密切关联,我们决定检测血清中IgA抗肌内膜抗体(EmA)。EmA呈阳性以及空肠活检显示全绒毛萎缩,从而确诊为CD。采用无麸质饮食后,患者临床症状显著改善,在3个月期间,对甲状腺和肾上腺替代疗法的需求逐渐减少。6个月后,血清EmA转阴,12个月后再次进行空肠活检显示黏膜完全恢复。无麸质饮食18个月后,抗甲状腺抗体滴度显著下降,我们得以停用甲状腺替代疗法。该病例强调了自身免疫性多腺体疾病与CD之间的关联;对这些病例的早期识别在临床上具有重要意义,这不仅是因为未治疗的CD存在并发症(如淋巴瘤)的高风险,还因为CD是自身免疫性甲状腺疾病患者替代激素治疗失败的原因之一。

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