Autoimmune polyglandular diseases

被引:32
作者
Kahaly, George J. [1 ]
Frommer, Lara [1 ]
机构
[1] Johannes Gutenberg Univ Mainz, Dept Med 1, Med Ctr, Mainz, Germany
关键词
autoimmune polyglandular disease; pathophysiology; familial clustering; psychosocial morbidity; management; REGULATORY T-CELLS; THYROID-STIMULATING IMMUNOGLOBULINS; IMMUNOLOGICAL SELF-TOLERANCE; CLINICAL-RELEVANCE; SYNDROME TYPE-2; GRAVES-DISEASE; ASSOCIATION; ANTIBODIES; AUTOANTIBODIES; POLYMORPHISM;
D O I
10.1016/j.beem.2019.101344
中图分类号
R5 [内科学];
学科分类号
1002 ; 100201 ;
摘要
Autoimmune polyglandular diseases (APD) are defined as the presence of two autoimmune -induced endocrine failures. With respect to the significant morbidity and potential mortality of APD, the diagnostic objective is to detect APD at an early stage, with the advantage of less frequent complications, effective therapy and better prognosis. This requires that patients at risk be regularly screened for subclinical endocrinopathies prior to clinical manifestation. Regarding the time interval between manifestation of first and further endocrinopathies, regular and long-term followup is warranted. Quality of life and psychosocial status are poor in APD patients and involved relatives. Familial clustering is high in patients with APD. Considering the high incidence of one or more endocrinopathies in first-degree relatives of patients with APD, family members should be regularly screened since they may also develop autoimmune endocrinopathies. Multidisciplinary management of these multiplex families in specialized centers is warranted. (C) 2019 Elsevier Ltd. All rights reserved.
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页数:12
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