Autoimmune polyendocrine syndromes.
نویسنده
چکیده
N Engl J Med 2004;350:2068-79. Copyright © 2004 Massachusetts Medical Society. he autoimmune polyendocrine syndromes are diverse, and their diversity is a characteristic that is both clinically important and instructive when their basic immunologic features are considered (Table 1). 1-4 These syndromes include monogenic disorders (such as autoimmune polyendocrine syndrome type I, which has classic and characteristic disease associations 5 ) and complex genetic disorders (such as autoimmune polyendocrine syndrome type II, in which the component diseases are more variable 6 ). Some of the component disorders are common (e.g., thyroid autoimmunity and celiac disease), whereas others are rare (e.g., Addison’s disease and myasthenia gravis). Some of the disorders are usually asymptomatic (e.g., celiac disease); others are symptomatic but typically diagnosed after years of illness (Addison’s disease, which features severe fatigue and nausea, and pernicious anemia, which causes neuropathic symptoms); and still others are clinically dramatic at the time of diagnosis (type 1A diabetes, also known as immune-mediated diabetes and formerly called insulin-dependent diabetes). The term “polyendocrine” itself is a misnomer, in that not all patients have multiple endocrine disorders, and many have nonendocrine autoimmune diseases. Nevertheless, the recognition that patients in whom multiple autoimmune disorders are diagnosed may have a specific genetic syndrome, may be at increased risk for multiple autoimmune disorders, and may have relatives who have an increased risk should spur clinicians toward early diagnosis and treatment. 7
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عنوان ژورنال:
- The New England journal of medicine
دوره 350 20 شماره
صفحات -
تاریخ انتشار 2004