scholarly journals Autoimmune polyglandular syndrome type 3

2020 ◽  
Vol 11 (4) ◽  
pp. 78-83
Author(s):  
G. R. Gazizova ◽  
M. R. Shaydullina ◽  
F. V. Valeeva ◽  
A. I. Galieva

Autoimmune polyglandular syndrome type 3 (APS type 3) is a combination of autoimmune thyroid damage in combination with one or more autoimmune endocrine or non-endocrine diseases. It is represented by three subtypes: APS 3A (graves ‘ Disease, or Hashimoto’s thyroiditis and type 1 diabetes mellitus), APS 3B (autoimmune thyroid disease and pernicious anemia), APS 3C (autoimmune thyroid disease and vitiligo, alopecia, and/or other organ-specific autoimmune diseases). Clinical observations of patients with type 3 APS are presented: characteristic clinical syndromes and clinical and laboratory characteristics of patients. These clinical examples demonstrate the importance of thorough examination, alertness and knowledge of endocrinologists about the possibility of developing polyglandular lesions of the endocrine system, the importance of timely prescribed treatment and interdisciplinary interaction of doctors.

2012 ◽  
Vol 97 (6) ◽  
pp. E1043-E1050 ◽  
Author(s):  
Ichiro Horie ◽  
Eiji Kawasaki ◽  
Takao Ando ◽  
Hironaga Kuwahara ◽  
Norio Abiru ◽  
...  

Objective: Type 1 diabetes (T1D) is commonly associated with autoimmune thyroid disease (AITD), and the occurrence of both T1D and AITD in a patient is defined as autoimmune polyglandular syndrome type 3 variant (APS3v). We aimed to clarify the differences in the clinical and genetic characteristics of APS3v patients and T1D patients without AITD [T1D/AITD(−)] in the Japanese population. Design/Patients: Our subjects were 54 APS3v patients and 143 T1D/AITD(−) patients who were consecutively diagnosed at Nagasaki University Hospital from 1983 to the present. Results: A remarkable female predominance, a slow and older age onset of T1D, and a higher prevalence of glutamic acid decarboxylase autoantibodies were observed in APS3v patients compared to T1D/AITD(−) patients. The older onset age of T1D in APS3v patients was associated with a higher proportion of slow-onset T1D. Among the two major susceptible human leukocyte antigen (HLA) class II haplotypes in Japanese T1D, DRB1*0405-DQB1*0401, but not DRB1*0901-DQB1*0303, was associated with APS3v patients. Furthermore, DRB1*0803-DQB1*0601 was not protective in patients with APS3v. The frequencies of the GG genotype in +49G>A and +6230G>A polymorphism in the CTLA4 gene were significantly higher in T1D/AITD(−) patients, but not in APS3v patients, compared to control subjects. Conclusions: In conclusion, we found notable differences in the clinical and genetic characteristics of APS3v patients and T1D/AITD(−) patients in the Japanese population, and the differences in the clinical characteristics between the two groups may reflect distinct genetic backgrounds including the HLA DRB1-DQB1 haplotypes and CTLA4 gene polymorphisms.


2011 ◽  
Vol 64 (3-4) ◽  
pp. 183-187 ◽  
Author(s):  
Ljiljana Todorovic-Djilas ◽  
Tijana Icin ◽  
Jovanka Novakovic-Paro ◽  
Ivana Bajkin

Introduction, Autoimmune diseases are chronic conditions initiated by the loss of immunological tolerance to self-antigens. They constitute heterogeneous group of disorders, in which multiple alterations in the immune system result in a spectrum of syndromes that either target specific organs or affect the body systematically. Recent epidemiological studies have shown a possible shift of one autoimmune disease to another or the fact that more than one autoimmune disease may coexist in a single patient or in the same family. Numerous autoimmune diseases have been shown to coexist frequently with thyroid autoimmune diseases. Autoimmune thyroid disease and other organ specific non-endocrine autoimmune diseases. This part of the study reviews the prevalence of autoimmune thyroid disease coexisting with: pernicious anaemia, vitiligo, celiac disease, autoimmune liver disease, miastenia gravis, alopecia areata and sclerosis multiplex, and several recommendations for screening have been given. Autoimmune thyroid disease and other organ non-specific non-endocrine autoimmune diseases. Special attention is given to the correlation between autoimmune thyroid disease and rheumatoid arthritis, systemic lupus erythematosus, syndrome Sj?gren, systemic sclerosis and mixed connective tissue disease. Conclusions. Screening for autoimmune thyroid diseases should be recommended in everyday clinical practice, in patients with primary organ-specific or organ non-specific autoimmune disease. Other?wise, in patients with primary thyroid autoimmune disease, there is no good reason of seeking for all other autoimmune diseases, although these patients have a greater risk of developing other autoimmune disease. Economic aspects of medicine require further analyzing of these data, from cost/benefit point of view to justified either mandatory screening or medical practitioner judgment.


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