«ОСОБЕННОСТИ ГОРМОНАЛЬНЫХ И ИММУННОЛОГИЧЕСКИХ НАРУШЕНИЙ У ПАЦИЕНТОВ С АУТОИММУННЫМ ПОЛИГЛАНДУЛЯРНЫМ СИНДРОМОМ»
Abstract
The aim of investigation is to evaluate the clinical and biochemical and immunological characteristics of patients with autoimmune polyglandular syndrome (APS) type 2.
Material and research methods. 258 patients were selected with an APS 2 of the type with the defeat of the endocrine glands. The following 3 subgroups of patients are formed: 1 gr. : with diabetes mellitus type 1 (DM 1)- 30 persons, 1b gr. - patients with primary adrenal insufficiency (PAI) - 32, 1B G. - patients with autoimmune thyroiditis (AIT) - 56 patients.
All studies that included generally clinical, biochemical, hormonal (ACTH, TSH, Cortisol, DHEA, insulin, free thyroxine), immunological research and ECG, ultrasound of internal organs, X-ray studies, consultation of narrow specialists, as well as statistical Methods performed all 188 patients. Immunological research methods included the determination in the blood of antibodies (AT) to the thyroid gland (AT TPO), to the adrenal glands (AT toN), to the pancreas (antibodies of glutamine acid decarboxylase (GAD / IA-2)), gender glands, antibodies to P450S21.
Research results. Patients prevailed in the age category from 18 to 44 years old, both among men and women - 58/35 cases, respectively.
As our studies have shown, serum levels of the CRH, the Russian Federation, MK and ATCCP in the group of patients with rheumatoid arthritis were significantly elevated in comparison with the control group
In patients with 1 A groups, the average levels of GAD / IA-2 were significantly elevated 16.7 ± 1.09 me / ml (p <0.05). The study of antibodies to P450S21 in the 1B group showed that the antibodies to P450C21 were detected in 23 (72%). In patients 1 in groups, the level of AT TPO was 26.8 ± 2.02 me / ml and was reliably clarified (p <0 05).
The study of antibodies to P450C21 in patients with PAI showed that antibodies to P450C21 were detected in 23 (72%) patients with PAI. In the comparison group in patients with CHNN tuberculosis genesis, the antibody to P450S21 was not detected.
Conclusions. 1. The immunological study of patients with autoimmune polyglandular syndrome is important in the early diagnosis of DM type1, AIT, and PAI diagnosis, in the individual assessment of the patient's compensation degree and should be included in the General Protocol for the conduct of patients in this category.
- The study of antibodies to P450S21 in the etiological diagnosis of PAI has a high diagnostic value, especially in the differential diagnosis of PAI tuberculosis genesis.
Введение. AПС представляет собой редкую полиэндокринопатию, характеризующуюся отказом нескольких желез внутренней секреции, а также неэндокринных органов, вызванной иммуноопосредованным разрушением тканей [5-8]. Это обычно приводит к гипофункциональному состоянию [11].
References
Мышкин К.И., Франкфурт Л.А. Аутоиммунитет и аутоиммуноагрессия //Аутоиммунные процессы в клинической хирургии.-Саратов, 1974.-Т. 104.- С. 122-125.
Панова Т.Н., Сучкова Е.Н. Особенности патологии щитовидной железы при аутоиммунных полиэндокринопатиях //Проблемы эндокринологии 1991.- Вып. 4.- С. 26-28.
Раскин А.М. Аутоиммунные процессы в патологии щитовидной железы /А.М. Раскин. Л: Медицина, 1973. - 222 с.
Сучкова Е.Н., Егорова С.П., Панова Т.Н. Течение сахарного диабета у больных синдромом аутоиммунной полиэндокринопатии: Материалы П Всероссийского съезда эндокринологов // Челябинск, 1991.-С. 174.
Dittmar M, Kahaly GJ. Polyglandular autoimmune syndromes: immunogenetics and long-term follow-up.//J Clin Endocrinol Metab 2003;88:2983–92
Hemminki K, Li X, Sundquist J & Sundquist K The epidemiology of Graves’ disease: evidence of a genetic and an environmental contribution. Journal of Autoimmunity 2010 34 J307–J313. (https://doi.org/10.1016/j.jaut.2009.11.019)
Hemminki K, Li X, Sundquist J & Sundquist K Risk of asthma and autoimmune diseases and related conditions in patients hospitalized for obesity. Annals of Medicine 2012 44 289–295. (https://doi.org/10.3109/07853890.2010.547515)
.Hemminki K, Liu X, Försti A, Sundquist J, Sundquist K & Ji J Subsequent Type 2 diabetes in patients with autoimmune disease. Scientific Reports 2015 5 13871. (https://doi.org/10.1038/srep13871)
Frommer Lara , George J Kahaly Autoimmune Polyendocrinopathy //J Clin Endocrinol Metab. 2019 Oct 1;104(10):4769-4782.doi: 10.1210/jc.2019-00602.
Gambineri Eleonora , Sara Ciullini Mannurita , David Hagin , Marina Vignoli Et all. Clinical, Immunological, and Molecular Heterogeneity of 173 Patients With the Phenotype of Immune Dysregulation, Polyendocrinopathy, Enteropathy, X-Linked (IPEX) Syndrome //Front Immunol. 2018 Nov 1;9:2411. doi: 10.3389/fimmu.2018.02411. eCollection 2018
Meyerson J, Lechuga-Gomez EE, Bigazzi PE, et al. Polyglandular autoimmune syndrome: current concepts.//CMAJ 2019;138:605–12.
Neufeld M, Blizzard RM. Polyglandular autoimmune disease. In: Pinchera A, Doniach D, FenziGF, Bachieri L, eds. Symposium on autoimmune aspects of endocrine disorders / New York: Academic Press. 1980; 357 – 26
Kahaly G J, L Frommer. Polyglandular autoimmune syndromes //J Endocrinol Invest. 2018 Jan;41(1):91-98. doi: 10.1007/s40618-017-0740-9. Epub 2017 Aug 17.
Krysiak Robert, Bogusław Okopień [Coexistence of autoimmune polyglandular syndrome type 3 with diabetes insipidus] //Wiad Lek 2015;68(2):204-7
Leonard MF. Chronic idiopathic hypoparathyroidism with superimposed Addison's disease in a child // J Clin Enocrinpl Metab 1946 -Vol.6 - P.493-495.
Marmont, A.M. Defining criteria for autoimmune diseases // Immunol Today 1994-Vol.15 - P. 388.
Mclntyre Gass JD. The syndrome of keratoconjunctivitis superficial moniliasis idiopathic hyporathyroidism and Addison's disease. Am J Ophthalmol.1962. 54 P.660-674.
McMahon FC, Cookson DV, Kabler JD, Inhorn SL. Idiopathic hypoparathyroidism and idiopathic adrenal cortical insufficiency occurring with cystic fibrosis of the pancreas // Ann Intern Med 1959 -Vol.51 - P.371-384.
Morse WI, Cochrane WA, Landigran PL. Familial hypoparathyroidism with pernicious anemia, steatorrhea and adrenocortical insufficiency // N Engl J Med 1961 - Vol.264 - P. 1021-1024.
Richman RA, Rosenthal IM, Solomon LM, Karachorlu KV. Candidiasis and multiple endocrinopathy with oral squamous cell carcinoma complications // Arch Dermatol 1975 - Vol.111 - P.625-627.
Varade J, Wang N, Lim CK, Zhang T, et al. Novel genetic loci associated HLA-B*08:01 positive myasthenia gravis. Journal of Autoimmunity 2018 88 43–
Wilgard Hunger-Battefeld 1, Katharina Fath, Alexandra Mandecka, Michael Kiehntopf, Christof Kloos. [Prevalence of polyglandular autoimmune syndrome in patients with diabetes mellitus type 1] //Med Klin (Munich). 2009 Mar 15; 104(3):183-91. doi: 10.1007/s00063-009-1030-x. Epub 2009 Apr 1.
