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

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, M_EF 68.9±6.6%, IU_Mm — 86 [75; 99] g, IU_SV — 60.9 [50; 66] ml, IU_EDVi — 52 [44; 59] ml/m² , IU_ESVi — 17 [15.3; 18] ml/m² , IU_ESV — 26 [23; 31] ml, IU_EDV — 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.

1. Larina AA, Troshina EA, Ivanova ON. Autoimmune polyglandular syndromes in the adults: the genetic and immunological diagnostic criteria. Problems of Endocrinology. 2014;60(3):43–52. (In Russ.). doi: 10.14341/probl201460343-52.

2. Kahaly GJ. Polyglandular autoimmune syndromes. Eur J Endocrinology. 2009;161(1):11−20. doi: 10.1530/eje09-0044.

3. Shulgina VYu, Fadeyev VV, Melnichenko GА. Myocardial lesion in hyperthyroidism: natural history, outcomes, prognosis. Clinical and experimental thyroidology. 2006;2(4):21−30. (In Russ.).

4. Marvisi M, Brianti M, Marani G, et al. Hyperthyroidism and pulmonary hypertension. Resp Med. 2002;96(4):215–220. doi: 10.1053/rmed.2001.1260.

5. Li JH, Safford RE, Aduen JF, et al. Pulmonary hypertension and thyroid disease. Chest. 2007;132(3):793–797. doi: 10.1378/chest.07-0366.

6. Moiseeva OM, Mitrofanova LB, Pahomov AV, et al. Spornyye voprosy diagnostiki miokarditov. Serdtse: zhurnal dlya praktikuyushchikh vrachey. 2010;9(4):234−241. (In Russ.).

7. Deryugin MV, Boytsov SA. Khronicheskiye miokardity. Saint Petersburg: ELBI-SPb; 2005. 288 р. (In Russ).

8. Kovalenko VN, Nesukai YeG, Cherniuk SV. Myocarditis: a contemporary look on the etiology and pathogenesis of the disease. Ukrainskii kardiologicheskii zhurnal. 2012;(2):84−91. (In Russ.).

9. Caforio AL, Goldman JH, Baig MK, et al. Cardiac autoantibodies in dilated cardiomyopathy become undetectable with disease progression. Heart. 1997;77(1):62–67. doi: 10.1136/hrt.77.1.62.

10. Poletaev AB. Novyye podkhody v rannem vyyavlenii patologicheskikh izmeneniy v organizme cheloveka (immunokhimicheskiy skrining kak osnova strategii perekhoda ot lechebnoy k preventivnoy meditsine). Metodicheskiye rekomendatsii dlya vrachey. 2nd ed. Moscow: MIC Immunkulus; 2011. 64 р. (In Russ.).

11. ACCF/ACR/AHA/NASCI/SCMR 2010 Expert Consensus Document on Cardiovascular Magnetic Resonance. J Am Coll Cardiol.2010;55(23):2614–2662. doi: 10.1016/j.jacc.2009.11.011.

12. Sezai A, Hata M, Minami K. Hypertrophic obstructive cardiomyopathy with Schmidt’s syndrome. Thorac Cardiov Surg. 2010;58(5):309−316. doi: 10.1055/s-0029-1185884.

13. Nielsen TD, Steenbergen C, Russell SD. Nonischemic cardiomyopathy associated with autoimmune polyglandular syndrome type II. Endocr Pract. 2005;13(1):59−62. doi: 10.4158/EP.13.1.59.

14. De Giorgi A, Fabbian F, Tiseo R, et al. Takotsubo cardiomyopathy and endocrine disorders: a mini-review of case reports. Am J Emerg. Med. 2014;32(11):1413−1417. doi: 10.1016/j.ajem.2014.07.030.

15. Cardiov KN, Chhabra L, Ahmad SA, et al. Case report: autoimmune polyglandular syndrome Type 2 complicated by acute adrenal crisis and pericardial tamponade in the setting of normal thyroid function. Methodist Debakey Cardiovasc J. 2015;11(4):250−252. doi: 10.14797/mdcj-11-4-250.

16. Campean R, Hasun M, Stöllberger C, et al. Takotsubo-like syndrome triggered by fludrocortisone overdose for Addison’s disease: a case report. J Med Case Rep. 2016;10(1):281. doi: 10.1186/s13256-016-1074-5.

17. Mochula OV. Magnitno-rezonansnaya tomografiya s kontrastnym usileniyem v otsenke povrezhdeniya miokarda pri nekoronarogennykh porazheniyakh serdtsa. [dissertation abstract] Tomsk; 2017. 27 р. (In Russ.).

18. Diagnosis and treatment of myocarditides. Draft clinical guidelines. Eurasian heart journal. 2019;(2):22−44. (In Russ.).

19. Mavrogeni S, Markussis V, Bratis K, et al. Hyperthyroidism induced autoimmune myocarditis. Evaluation by cardiovascular magnetic resonance and endomyocardial biopsy. Int J Cardiol. 2012;158(1):166−168. doi: 10.1016/j.ijcard.2012.04.089.

20. Fatourechi V, Edwards WD. Graves’ disease and low‐output cardiac dysfunction: implications for autoimmune disease in endomyocardial biopsy tissue from eleven patients. Thyroid. 2000;10(7):601−605. doi: 10.1089/thy.2000.10.601.