[Instrumental and laboratory parameters of myocardial function in adult patients with autoimmune polyglandular syndrome type 2, 3]

Probl Endokrinol (Mosk). 2020 Sep 25;66(4):16-23. doi: 10.14341/probl12554.
[Article in Russian]


Backgraund: Аutoimmune polyglandular syndrome (APS) it is characterized by damage to two or more endocrine glands, which eventually results in the hormonal failure. Some clinical studies describe the development of myocardial lesion in the setting of combined autoimmune endocrine pathology. In Russia the myocardial condition in adult patients with APS types 2 and 3 was examined for the first time.

Aim: To evaluate the structure and functional state of the myocardium according to magnetic resonance imaging (MRI), to analyze changes in the spectrum of specific antiheart autoantibodies and markers of heart lesion in patients with APS types 2 and 3.

Materials and methods: 50 patients with APS types 2, 3 were studied. 45 of them were performed with delayed contrast heart MRI. All 50 patients were tested for IgG antibodies to heart muscle antigens by indirect enzymatic immunoassay (EIA), for troponin I and natriuretic peptide by chemiluminescence immunoassay (CLIA), for creatine phosphokinase (CPK) by NAC (N - acetyl-L-cysteine), and for C-reactive protein (CRP) by immunoturbidimetry.

Results: According to the results of heart MRI (n=45), 91% showed signs of functional changes in the left ventricular (LV) myocardium without any signs of myocarditis. 38 of 45 examined patients had deviation of 2 or more indicants of the LV functional state, MEF 68.9±6.6%, IUMm - 86 [75; 99] g, IUSV - 60.9 [50; 66] ml, IUEDVi - 52 [44; 59] ml/m2 , IUESVi - 17 [15.3; 18] ml/m2 , IUESV - 26 [23; 31] ml, IUEDV - 85 [70; 92] ml. 1 patient (2%) had positive result according to the determination of antibodies (AB) to heart muscle antigens (AG). Troponin 1 indicants did not exceed the reference values. The level of CPK exceeded the reference values in 3 patients (6%), an increase of CRP, NT-proBNP was observed in 7 patients (14%), and a combined increase was observed in 1 case.

Conclusions: We obtained MRI data indicating functional changes in the myocardium in patients with APS types 2 and 3. The autoimmune cause of these changes according to the results of determining of antiheart antibodies was not confirmed in most of the examined patients, the indicants of «damage» to the myocardium (troponin 1 and NT-proBNP) did not deviate from the reference range.

MeSH terms

  • Adult
  • Heart / diagnostic imaging
  • Humans
  • Laboratories
  • Myocarditis*
  • Myocardium
  • Polyendocrinopathies, Autoimmune*