Features of clinical manifestations and treatment of Schmidt’s syndrome

Main Article Content

L.V. Shkala

Abstract

Background. Autoimmune polyglandular syndrome type 2 is the most common. The development and course of Schmidt’s syndrome is of interest, which requires a comprehensive diagnosis due to the combination of several autoimmune endocrinopathies, and rational therapy. The purpose of this work was to analyze the features of clinical manifestations and treatment of autoimmune polyglandular syndrome type 2 using a specific example. Materials and methods. A clinical case of Schmidt’s syndrome in a 36-year-old female patient is presented for consideration. There were primary adrenal insufficiency with an autoimmune process to 21-hydroxylase and the presence of autoimmune thyroiditis with the development of hypothyroidism. Results. The clinical manifestations of the disease were mainly represented by syndromes of hypotension, general and muscle weakness, hyperpigmentation, weight loss, dyspeptic disorders, aggravated by secondary viral infection, against the background of edematous syndrome. Additional examination revealed a significant increase in adrenocorticotropic hormone, renin, hyperkalemia, hypercholesterolemia, increased 17-hydroxyprogesterone, thyroid-stimulating hormone, hypothyroxinemia, an increase in antibodies to 21-hydroxylase and thyroid peroxidase and a decrease in the thyroid volume with multiple linear inclusions, increased echogenicity. The analysis of the identified hormonal, metabolic and pathomorphological changes made it possible to establish the pre­sence of Schmidt’s syndrome in this clinical case. The treatment with glucocorticoids was accompanied by adverse reactions, which led to repeated replacement of drugs. The choice was made in favor of сortef, the dose was carefully titrated under the control of cortisoluria. The administration of levothyroxine was accompanied by the achievement of euthyroidism. Conclusions. The symptoms of Schmidt’s syndrome with the simultaneous manifestation of primary adrenal insufficiency of autoimmune origin and autoimmune thyroiditis with the deve­lopment of hypothyroidism differed in terms of a mutually aggravating course. There were significant difficulties in the treatment of this polyglandular syndrome associated with the correction of hypocorticism due to poor tolerance of glucocorticoids, which required constant monitoring, continuous control of the hormonal and metabolic status.

Article Details

How to Cite
Shkala, L. “Features of Clinical Manifestations and Treatment of Schmidt’s Syndrome”. INTERNATIONAL JOURNAL OF ENDOCRINOLOGY (Ukraine), vol. 16, no. 8, Aug. 2021, pp. 713-20, doi:10.22141/2224-0721.16.8.2020.222894.
Section
Clinical Case

References

Vorobyev SV, Khripun IA, Kuzmenko NA, Streltsova EM, Petrovskaya EY. Schmidt syndrome in clinical practice. South Russian Journal of Therapeutic Practice. 2020;1(2):88-92. doi:10.21886/2712-8156-2020-1-2-88-92. (in Russian).

Husebye ES, Anderson MS, Kämpe O. Autoimmune Polyendocrine Syndromes. N Engl J Med. 2018 Mar 22;378(12):1132-1141. doi:10.1056/NEJMra1713301.

Gutierrez MJ, Gilson J, Zacharias J, Ishmael F, Bingham CA. Childhood Polyarthritis As Early Manifestation of Autoimmune Polyendocrinopathy with Candidiasis and Ectodermal Dystrophy Syndrome. Front Immunol. 2017 Apr 18;8:377. doi:10.3389/fimmu.2017.00377.

Kakleas K, Soldatou A, Karachaliou F, Karavanaki K. Associated autoimmune diseases in children and adolescents with type 1 diabetes mellitus (T1DM). Autoimmun Rev. 2015 Sep;14(9):781-797. doi:10.1016/j.autrev.2015.05.002.

Lebovitz HE. Autoimmune polyglandular syndromes: interplay between the immune and the endocrine systems leading to a diverse set of clinical diseases and new insights into immune regulation. Diabetes Technol Ther. 2013 Jun;15 Suppl 2:S2-21-S2-28. doi:10.1089/dia.2013.0130.

Azad AK, Islam MS, Quayum SL. Autoimmune polyglandular syndrome type II - a case report. Mymensingh Med J. 2015 Jan;24(1):199-201.

Gendeleka GF, Gendeleka AN. Difficulties in the diagnosis and therapeutic approach in autoimmune polyglandular syndrome type 2. A clinical сase. Mìžnarodnij endokrinologìčnij žurnal. 2018;14(1):99-102. doi:10.22141/2224-0721.14.1.2018.127101. (in Russian).

Bornstein SR, Allolio B, Arlt W, et al. Diagnosis and Treatment of Primary Adrenal Insufficiency: An Endocrine Society Clinical Practice Guideline. J Clin Endocrinol Metab. 2016 Feb;101(2):364-389. doi:10.1210/jc.2015-1710.

Karamifar H, Dalili S, Karamizadeh Z, Amirhakimi G, Dalili H. Autoimmune polyglandular syndrome type 2: an unusual presentation. Acta Med Iran. 2010 May-Jun;48(3):196-197.

Smith RK, Gerrits PM. A Rare Case of Autoimmune Polyglandular Syndrome Type 2 in a Child With Persistent Fatigue. Glob Pediatr Health. 2019 May 1;6:2333794X19845074. doi:10.1177/2333794X19845074.

Gouda MR, Al-Amin A, Grabsch H, Donnellan C. A multidisciplinary approach to management of autoimmune polyendocrinopathy candidiasis ectodermal dystrophy (APECED). BMJ Case Rep. 2013 Jan 30;2013:bcr2012008116. doi:10.1136/bcr-2012-008116.

Wang X, Ping F, Qi C, Xiao X. Delayed diagnosis with autoimmune polyglandular syndrome type 2 causing acute adrenal crisis: A case report. Medicine (Baltimore). 2016 Oct;95(42):e5062. doi:10.1097/MD.0000000000005062.

Brandão Neto RA, de Carvalho JF. Diagnosis and classification of Addison's disease (autoimmune adrenalitis). Autoimmun Rev. 2014 Apr-May;13(4-5):408-11. doi:10.1016/j.autrev.2014.01.025.

Meyer G, Badenhoop K, Linder R. Addison's disease with polyglandular autoimmunity carries a more than 2·5-fold risk for adrenal crises: German Health insurance data 2010-2013. Clin Endocrinol (Oxf). 2016 Sep;85(3):347-353. doi:10.1111/cen.13043.

Dittmar M, Kahaly GJ. Polyglandular autoimmune syndromes: immunogenetics and long-term follow-up. J Clin Endocrinol Metab. 2003 Jul;88(7):2983-2992. doi:10.1210/jc.2002-021845.