Schmidt syndrome – is it easy to recognize? Case report
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Student Research Group, Endocrinology and Diabetology Clinic, Medical University, Lublin, Poland
Department of Endocrinology and Diabetology, Medical University, Lublin, Poland
Magdalena Kozioł   

Student Research Group, Endocrinology and Diabetology Clinic, Medical University, Lublin, Poland
Med Og Nauk Zdr. 2022;28(4):341–344
Schmidt›s syndrome is one of the APS-2 variants, occurring with the frequency of 1.5–4.5 / 100,000, diagnosed approximately twice as often in women. Family occurrence is observed. Schmidt›s syndrome is found in the form of Addison›s disease and autoimmune thyroid disease. The units that make up the syndrome can appear in any order and time, and overlapping symptoms make diagnosis difficult.

Case Report:
A 39-year-old patient with hypothyroidism and impending adrenal crisis was admitted to the Endocrinology Department for hormonal evaluation. In the history of about a year, weight loss of 20 kg, lack of appetite, weakness, hypotonia, slightly darker skin with a large number of freckles. Second amenorrhea for a year and symptoms of depression. Two weeks earlier, the patient was hospitalized in the neurology department due to confusion, hyponatremia and cerebral edema found on CT. Symptoms after administration of mannitol and hydrocortisone resolved. Moreover, the abdominal CT scan revealed hypotrophic adrenal glands. In the Department of Endocrinology, a low concentration of morning cortisol was confirmed with a high level of ACTH (1623 pg / ml), which allowed confirmation of the diagnosis of Addison‘s disease. In addition, Hashimoto‘s disease was found. The coexistence of both diseases allowed the diagnosis of Schmidt‘s syndrome.

The probability that a patient with one autoimmune disease will be diagnosed with another entity from the autoimmune spectrum is very high. The presented patient had typical symptoms of adrenal insufficiency, but they were associated with malnutrition, hypothyroidism and depression. Often, the symptoms of Addison‘s disease are masked by hypothyroidism, which makes diagnosis difficult.

Bain A, Stewart M, Mwamure P, et al. Addison›s disease in a patient with hypothyroidism: autoimmune polyglandular syndrome type 2. BMJ Case Rep. 2015; 2015:bcr2015210506. doi: 10.1136/bcr-2015-210506.
Resende E, Gómez GN, Nascimento M, et al. Precocious presentation of autoimmune polyglandular syndrome type 2 associated with an AIRE mutation. Hormones (Athens). 2015;14(2):312–6. doi:10.14310/ horm.2002.1513.
Schmidt MB. Eine biglandulare Erkrankung (Nebennieren und Schilddrüse) bei Morbus Addisonii. Verh Dtsch Ges Pathol 1926;21:212–21.
Wells HG. Addison›s disease with the selective destruction of the suprarenal cortex. Arch Pathol. 1930;10:499–523.
Smith RK, Gerrits PM. A Rare Case of Autoimmune Polyglandular Syndrome Type 2 in a Child With Persistent Fatigue. Glob Pediatr Health. 2019;1;6:2333794X19845074. doi: 10.1177/2333794X19845074.
Kahaly GJ, Frommer L. Autoimmune polyglandular diseases. Best Pract Res Clin Endocrinol Metab. 2019;33(6):101344. doi: 10.1016/j.beem.2019.101344.
Kahaly GJ, Frommer L. Polyglandular autoimmune syndromes. J Endocrinol Invest. 2018;41,91–98.
Schulz L, Hammer E. Autoimmune polyglandular syndrome type II with co-manifestation of Addison›s and Graves› disease in a 15-yearold boy: case report and literature review. J Pediatr Endocrinol Metab. 2020;28;33(4):575–578. doi: 10.1515/jpem-2019-0506.
Anglin RE, Rosebush PI, Mazurek MF. The neuropsychiatric profile of Addison›s disease: revisiting a forgotten phenomenon. J Neuropsychiatry Clin Neurosci. 2006;18(4):450–9. doi: 10.1176/jnp.2006.18.4.450.
Patel DM, Gurumikhani JK, Patel MV, et. al. Adrenocortical Crisis Triggered by Levothyroxine in an Unrecognized Autoimmune Polyglandular Syndrome Type-2: A Case Report with Review of the Literature. Curr Drug Saf. 2021;16(1):101–106. doi: 10.2174/15748863 15666200826095842.
Anyfantakis D, Symvoulakis EK, Vourliotaki I, et al. Schmidt's syndrome presenting as a generalised anxiety disorder: a case report. J Med Life. 2013;6(4):451–3.
Barreda-Velit C, Salcedo-Pereda R, Ticona J. Síndrome de Schmidt: diagnóstico difícil en el contexto latinoamericano [Schmidt’s syndrome: a difficult diagnosis in the Latin American context]. Rev Med Inst Mex Seguro Soc. 2018;56(2):189–193.
Gupta AN, Nagri SK. Schmidt's syndrome – Case report. Australas Med J. 2012;5(6):292–5. doi: 10.4066/AMJ.2012.987.
Gaba N, Gaba S, Singla M, et. al. Type 2 Autoimmune Polyglandular Syndrome Presenting with Hyperpigmentation and Amenorrhea. Cureus. 2020;12(4): e7772. doi: 10.7759/cureus.7772.
Saif A, Assem M. Premature ovarian failure could be an alarming sign of polyglandular autoimmune dysfunction. Endocr Regul. 2017;25;51(2):114–116. doi: 10.1515/enr-2017-0011.
Yanachkova V, Chaveeva P, Staynova R, et al. Autoimmune Polyglandular Syndrome Type 2 and Pregnancy. Folia Med (Plovdiv) 2020;30;62(2):408–411. doi: 10.3897/folmed.62.e48580. PMID: 32666756.
Stahn B, Scheit L. Das Schmidt-Syndrom [The Schmidt's Syndrome]. Dtsch Med Wochenschr. 2019;144(24):1741–1744. doi: 10.1055/a-0875-4538. PMID: 31791082.
Wiśniewski OW, Matuszak P, Kasprzak A, et al. Severe hyponatremia as the first sign of late-onset Schmidt's syndrome: A case report. Caspian J Intern Med. 2021;12(Suppl 2):S392-S396. doi: 10.22088/cjim.12.0.392.
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;95(42):e5062. doi: 10.1097/ MD.0000000000005062.
De-Giorgio F, Foti F, Bergamin E, et al. Regarding „Fatal adrenal crisis due to Addison›s disease arising in the context of autoimmune polyglandularsyndrome type 1”. Forensic Sci Med Pathol. 2022;18(2):226–227. doi:10.1007/s12024-021-00439-0.
Flesch BK, König J, Frommer L, et al. Gender alters the MHC class I HLA-A association with polyglandular autoimmunity. J Clin Endocrinol Metab. Figshare 2018. Deposited 25 October 2018. https://