Autoimmune and Autoinflammatory Genesis of Myocarditis and Pericarditis

Cover Page


Cite item

Full Text

Open Access Open Access
Restricted Access Access granted
Restricted Access Subscription or Fee Access

Abstract

Myocarditis and pericarditis are common pathologies in the general population, which pose significant challenges to practitioners for both diagnosis and treatment due to the unique characteristics of diagnosis verification and lack of clear understanding of the diseases’ pathogenesis. This publication discusses the primary mechanisms of inflammatory diseases’ development of the myocardium and pericardium based on innate and acquired immunity disorders, including data from our research confirming the autoimmune and autoinflammatory nature of these diseases. The paper presents the main clinical manifestations and laboratory markers that enable differential diagnosis between autoimmune and autoinflammatory heart diseases. Additionally, it provides an evidence-based approach to the effectiveness of various anti-inflammatory and immunosuppressive drugs in different types and stages of inflammatory diseases of the myocardium and pericardium.

Full Text

Restricted Access

About the authors

Valentina Yu. Myachikova

Almazov National Medical Research Centre

Email: myachikova_vyu@almazovcentre.ru
ORCID iD: 0000-0002-7590-742X
SPIN-code: 8874-1824

MD

Russian Federation, St. Petersburg

Alexey L. Maslyanskiy

Almazov National Medical Research Centre

Email: esc_4@mail.ru
ORCID iD: 0000-0003-2427-4148
SPIN-code: 5698-7797

MD, PhD, Professor

Russian Federation, St. Petersburg

Sofia V. Mairina

Almazov National Medical Research Centre

Email: sonya.spbgmu16@gmail.com
ORCID iD: 0000-0002-0015-8620
SPIN-code: 9900-7817

MD

Russian Federation, St. Petersburg

Lubov B. Mitrofanova

Almazov National Medical Research Centre

Email: lubamitr@yandex.ru
ORCID iD: 0000-0003-0735-7822
SPIN-code: 9552-8248

MD, PhD, Assistant Professor

Russian Federation, St. Petersburg

Anna A. Kostareva

Almazov National Medical Research Centre

Email: akostareva@hotmail.com
ORCID iD: 0000-0002-9349-6257
SPIN-code: 5343-6287

MD, PhD, Assistant Professor

Russian Federation, St. Petersburg

Olga M. Moiseeva

Almazov National Medical Research Centre

Author for correspondence.
Email: moiseeva@almazovcentre.ru
ORCID iD: 0000-0002-7817-3847
SPIN-code: 1492-3900

MD, PhD

Russian Federation, St. Petersburg

References

  1. Dai H, Lotan D, Much AA, et al. Global, Regional, and National Burden of Myocarditis and Cardiomyopathy, 1990–2017. Front Cardiovasc Med. 2021;8:610989. doi: https://doi.org/10.3389/fcvm.2021.610989
  2. Митрофанова Л.Б., Макаров И.А., Рунов А.Л., и др. Клинико-морфологическое и молекулярно-биологическое исследование миокарда у пациентов с COVID-19 // Российский кардиологический журнал. — 2022. — Т. 27. — № 7. — С. 4810. [Mitrofanova LB, Makarov IA, Runov AL, et al. Clinical, morphological and molecular biological examination of the myocardium in COVID-19 patients. Russian Journal of Cardiology. 2022;27(7):4810. (In Russ.)] doi: https://doi.org/10.15829/1560-4071-2022-4810
  3. Cavalli G, Colafrancesco S, Emmi G, et al. Interleukin 1α: a comprehensive review on the role of IL-1α in the pathogenesis and treatment of autoimmune and inflammatory diseases. Autoimmun Rev. 2021;20(3):102763. doi: https://doi.org/10.1016/j.autrev.2021.102763
  4. McGonagle D, McDermott MF. A proposed classification of the immunological diseases. PLoS Med. 2006;3(8):e297. doi: https://doi.org/10.1371/journal.pmed.0030297
  5. Ehrlich P. The collected papers of Paul Ehrlich. New York; 1957.
  6. Dameshek W, Schwartz SO. Hemolysins as the cause of clinical and experimental hemolytic anemias. With particular reference to the nature of spherocytosis and increased fragility. The American Journal of the Medical Sciences. 1938;196(12):769–789.
  7. Rose NR, Bona C. Defining criteria for autoimmune diseases (Witebsky’s postulates revisited). Immunology Today. 1993;14(9):426–430. doi: https://doi.org/10.1016/0167-5699(93)90244-F
  8. Roitt IM, Doniach D, Campbell PN. Auto-antibodies in Hashimoto’s disease (lymphadenoid goitre). Lancet. 1956;271(6947):820–821. doi: https://doi.org/10.1016/s0140-6736(56)92249-8
  9. Abbas AK, Lichtman AH, Pillai S. Basic Immunology: Functions and Disorders of the Immune System. 6th ed. Philadelphia: Elsevier; 2019. 336 p.
  10. McDermott MF, Aksentijevich I, Galon J, et al. Germline mutations in the extracellular domains of the 55 kDa TNF receptor, TNFR1, define a family of dominantly inherited autoinflammatory syndromes. Cell. 1999;97(1):133–144. doi: https://doi.org/10.1016/s0092-8674(00)80721-7
  11. Latz E, Xiao TS, Stutz A. Activation and regulation of the inflammasomes. Nat Rev Immunol. 2013;13(6):397–411. doi: https://doi.org/10.1038/nri3452
  12. Van Linthout S, Tschöpe C. Inflammation — Cause or Consequence of Heart Failure or Both? Curr Heart Fail Rep. 2017;14(4):251–265. doi: https://doi.org/10.1007/s11897-017-0337-9
  13. Рунов А., Курчакова Е., Хащевская Д., и др. Экспрессия вирусных рецепторов и развитие миокардита // Труды Института полиомиелита и вирусных инфекций имени М.П. Чумакова РАМН. Медицинская вирусология. — 2016. — Т. 30. — № 1. — С. 39–47. [Runov A, Kurchakova E, Hashchevskaya D, i dr. Ekspressiya virusnyh receptorov i razvitie miokardita. Trudy Instituta poliomielita i virusnyh infekcij imeni M.P. Chumakova RAMN. Medicinskaya virusologiya. 2016;30(1):39–47. (In Russ.)]
  14. Swirski FK, Nahrendorf M. Cardioimmunology: the immune system in cardiac homeostasis and disease. Nat Rev Immunol. 2018;18(12):733–744. doi: https://doi.org/10.1038/s41577-018-0065-8
  15. Hintenberger R, Falkinger A, Danninger K, et al. Cardiovascular disease in patients with autoinflammatory syndromes. Rheumatol Int. 2018;38(1):37–50. doi: https://doi.org/10.1007/s00296-017-3854-7
  16. ter Haar NM, Oswald M, Jeyaratnam J, et al. Recommendations for the management of autoinflammatory diseases. Ann Rheum Dis. 2015;74(9):1636–1644. doi: https://doi.org/10.1136/annrheumdis-2015-207546
  17. Efthimiou P, Petryna O, Nakasato P, et al. New insights on multigenic autoinflammatory diseases. Ther Adv Musculoskelet Dis. 2022;14:1759720X221117880. doi: https://doi.org/10.1177/1759720X221117880
  18. Fautrel B. Adult-onset Still disease. Best Pract Res Clin Rheumatol. 2008;22(5):773–792. doi: https://doi.org/10.1016/j.berh.2008.08.006
  19. Tschöpe C, Ammirati E, Bozkurt B, et al. Myocarditis and inflammatory cardiomyopathy: current evidence and future directions. Nat Rev Cardiol. 2021;18(3):169–193. doi: https://doi.org/10.1038/s41569-020-00435-x
  20. Хащевская Д.А., Митрофанова Л.Б., Белякова Е.А., и др. Аутоиммунитет и прогноз больных с морфологически документированным миокардитом // Кардиология. — 2016. — № 6. — С. 50–57. [Hashchevskaya DA, Mitrofanova LB, Belyakova EA, i dr. Autoimmunitet i prognoz bol’nyh s morfologicheski dokumentirovannym miokarditom. Kardiologiya. 2016;6:50–57. (In Russ.)] doi: https://doi.org/10.18565/cardio.2016.6.50-57
  21. Şelli ME, Thomas AC, Wraith DC, et al. A humanized HLA-DR4 mouse model for autoimmune myocarditis. J Mol Cell Cardiol. 2017;107:22–26. doi: https://doi.org/10.1016/j.yjmcc.2017.04.003
  22. Błyszczuk P. Myocarditis in Humans and in Experimental Animal Models. Front Cardiovasc Med. 2019;6:64. doi: https://doi.org/10.3389/fcvm.2019.00064
  23. Майрина С.В., Титов В. А., Митрофанова Л.Б., и др. Анализ эффективности иммуносупрессивной терапии лимфоцитарного миокардита по данным реальной клинической практики // Российский кардиологический журнал. — 2021. — Т. 26. — № 11. — С. 4696. [Mairina SV, Titov VA, Mitrofanova LB, et al. Effectiveness of immunosuppressive therapy for lymphocytic myocarditis according: data from actual clinical practice. Russian Journal of Cardiology. 2021;26(11):4696. (In Russ.)] doi: https://doi.org/10.15829/1560-4071-2021-4696
  24. Artico J, Merlo M, Delcaro G, et al. Lymphocytic Myocarditis: A Genetically Predisposed Disease? J Am Coll Cardiol. 2020;75(24):3098–3100. doi: https://doi.org/10.1016/j.jacc.2020.04.048
  25. Kontorovich AR, Patel N, Moscati A, et al. Myopathic Cardiac Genotypes Increase Risk for Myocarditis. JACC Basic Transl Sci. 2021;6(7):584–592. doi: https://doi.org/10.1016/j.jacbts.2021.06.001
  26. Adler Y., Charron P., Imazio M., и др. Рекомендации ESC по диагностике и ведению пациентов с заболеваниями перикарда 2015 // Российский кардиологический журнал. — 2016. — Т. 21. — № 5. — С. 117–162. [Adler Y, Charron P, Imazio M, et al. 2015 ESC Guidelines for the Diagnosis and Management of Pericardial Diseases. Russian Journal of Cardiology. 2016;(5):117–162. (In Russ.)] doi: https://doi.org/10.15829/1560-4071-2016-5-117-162
  27. Мячикова В.Ю., Маслянский А.Л., Ткаченко О.Ю., и др. Сравнительный анализ концентраций провоспалительных цитокинов и гликозилированного ферритина у пациентов с идиопатическим рецидивирующим перикардитом и болезнью Стилла взрослых // Российский кардиологический журнал. — 2021. — Т. 26. — № 11. — С. 4715. [Myachikova VYu, Maslyansky AL, Tkachenko OYu, et al. Comparative analysis of the concentrations of proinflammatory cytokines and glycosylated ferritin in patients with idiopathic recurrent pericarditis and adult-onset Still’s disease. Russian Journal of Cardiology. 2021;26(11):4715. (In Ryss.)] doi: https://doi.org/10.15829/1560-4071-2021-4715
  28. Brucato A, Imazio M, Gattorno M, et al. Effect of Anakinra on Recurrent Pericarditis Among Patients with Colchicine Resistance and Corticosteroid Dependence: The AIRTRIP Randomized Clinical Trial. JAMA. 2016;316(18):1906–1912. doi: https://doi.org/10.1001/jama.2016.15826
  29. Klein AL, Imazio M, Cremer P, et al. RHAPSODY Investigators. Phase 3 Trial of Interleukin-1 Trap Rilonacept in Recurrent Pericarditis. N Engl J Med. 2021;384(1):31–41. doi: https://doi.org/10.1056/NEJMoa2027892
  30. Mauro AG, Bonaventura A, Vecchié A, et al. The Role of NLRP3 Inflammasome in Pericarditis: Potential for Therapeutic Approaches. JACC Basic Transl Sci. 2021;6(2):137–150. doi: https://doi.org/10.1016/j.jacbts.2020.11.016
  31. Rodolfi S, Nasone I, Folci M, et al. Autoinflammatory Manifestations in Adult Patients. Clin Exp Immunol. 2022;210(3):295–308. doi: https://doi.org/10.1093/cei/uxac098
  32. Vitale A, Sota J, Obici L, et al. Role of Colchicine Treatment in Tumor Necrosis Factor Receptor Associated Periodic Syndrome (TRAPS): Real-Life Data from the AIDA Network. Mediators Inflamm. 2020;2020:1936960. doi: https://doi.org/10.1155/2020/1936960
  33. Lazaros G, Vlachopoulos C, Lazarou E, et al. New Approaches to Management of Pericardial Effusions. Curr Cardiol Rep. 2021;23(8):106. doi: https://doi.org/10.1007/s11886-021-01539-7
  34. Vianello F, Cinetto F, Cavraro M, et al. Azathioprine in isolated recurrent pericarditis: a single centre experience. Int J Cardiol. 2011;147(3):477–478. doi: https://doi.org/10.1016/j.ijcard.2011.01.027
  35. Toldo S, Kannan H, Bussani R, et al. Formation of the inflammasome in acute myocarditis. Int J Cardiol. 2014;171(3):e119–e121. doi: https://doi.org/10.1016/j.ijcard.2013.12.137
  36. Abbate A, Toldo S, Marchetti C, et al. Interleukin-1 and the Inflammasome as Therapeutic Targets in Cardiovascular Disease. Circ Res. 2020;126(9):1260–1280. doi: https://doi.org/10.1161/CIRCRESAHA.120.315937
  37. Paraskevaidis I, Farmakis D, Papingiotis G, et al. Inflammation and Heart Failure: Searching for the Enemy-Reaching the Entelechy. J Cardiovasc Dev Dis. 2023;10(1):19. doi: https://doi.org/10.3390/jcdd10010019

Supplementary files

Supplementary Files
Action
1. JATS XML
Download
2. Figure 1. Autoantibody profile in patients with morphologically documented myocarditis compared with a control group of apparently healthy patients. Autoantibodies to cardiomyocyte proteins: MyBPC3 — to myosin-binding protein; ANT-1 and ANT-2 - to two peptide fragments of the protein - adenine nucleotide translocator, a fragment of the extracellular loop of the β1-adrenergic receptor; protein p53, CoM-02 - to proteins of the cytoskeleton of cardiomyocytes; CoS-05-40 - to cytoplasmic proteins, cardiac myosin, collagen; M2 - to cholinergic receptors

Download (192KB)
Indexing metadata
3. Figure 2. Cumulative survival and need for heart transplantation in patients with morphologically documented lymphocytic myocarditis depending on the prescription of glucocorticosteroids (n = 105, of which 36 patients received immunosuppressive therapy) (Long-rank p = 0.0032)

Download (150KB)
Indexing metadata
4. Figure 3. Characteristics of pericarditis by duration, pathogenesis and nosologies

Download (380KB)
Indexing metadata

Copyright (c) 2023 "Paediatrician" Publishers LLC



This website uses cookies

You consent to our cookies if you continue to use our website.

About Cookies