一名患有3型多腺体自身免疫综合征的患者出现自身免疫性自发性慢性荨麻疹和全身性重症肌无力。
Autoimmune spontaneous chronic urticaria and generalized myasthenia gravis in a patient with polyglandular autoimmune syndrome type 3.
作者信息
De Marchi Sergio Umberto, Cecchin Emanuela, De Marchi Sergio
机构信息
Unit of Internal Medicine, Department of Experimental and Clinical Medicine, Santa Maria della Misericordia Academic Hospital, University of Udine, Via Colugna 50, 33100 Udine, Italy.
出版信息
Muscle Nerve. 2015 Sep;52(3):440-4. doi: 10.1002/mus.24635.
INTRODUCTION
Autoimmune polyglandular syndromes (APS) are a group of disorders characterized by the presence of autoimmune processes in several endocrine and non-endocrine organs that are classified into 4 types.
METHODS
We describe the case of a woman affected with APS type 3 who presented initially with Hashimoto thyroiditis and pernicious anemia and subsequently developed autoimmune chronic urticaria, myasthenia gravis, and type 1 diabetes mellitus.
RESULTS
This patient had a combination of components of APS types 3b, 3a, and 3c. She was referred for evaluation of chronic spontaneous urticaria and subsequently developed severe generalized myasthenia gravis, which was apparently unmasked by antihistamines used to control urticaria.
CONCLUSIONS
Patients with APS should have a more thorough evaluation to better clarify their autoimmune background. Early detection of autoantibodies and latent organ-specific dysfunction may help physicians take appropriate action to prevent full-blown disease.
引言
自身免疫性多腺体综合征（APS）是一组疾病，其特征是在多个内分泌和非内分泌器官中存在自身免疫过程，分为4种类型。
方法
我们描述了一名患有3型APS的女性病例，该患者最初表现为桥本甲状腺炎和恶性贫血，随后发展为自身免疫性慢性荨麻疹、重症肌无力和1型糖尿病。
结果
该患者具有3b型、3a型和3c型APS的多种成分组合。她因慢性自发性荨麻疹前来评估，随后发展为严重的全身性重症肌无力，这显然是由用于控制荨麻疹的抗组胺药引发的。
结论
APS患者应进行更全面的评估，以更好地明确其自身免疫背景。早期检测自身抗体和潜在的器官特异性功能障碍可能有助于医生采取适当措施预防疾病全面发作。
Zotero 插件