Myocarditis as the first manifestation of eosinophilic granulomatosis with polyangiitis

Danijela Djordjević Radojković; Svetlana  Apostolović; Miodrag Damjanović; Tomislav Kostić; Aleksandra Fejsa Levakov; Marko Dimitrijević; Ružica Janković Tomašević; Sonja Dakić; Nenad Božinović; Milena Pavićević

doi:10.2298/VSP230218026D

Danijela Djordjević Radojković University Clinical Center Niš, Cardiology Clinic, Niš, Serbia; University of Niš, Faculty of Medicine, Niš, Serbia
Svetlana Apostolović University Clinical Center Niš, Cardiology Clinic, Niš, Serbia; University of Niš, Faculty of Medicine, Niš, Serbia
Miodrag Damjanović University Clinical Center Niš, Cardiology Clinic, Niš, Serbia
Tomislav Kostić University Clinical Center Niš, Cardiology Clinic, Niš, Serbia; University of Niš, Faculty of Medicine, Niš, Serbia
Aleksandra Fejsa Levakov University of Novi Sad, Faculty of Medicine, Novi Sad, Serbia; University Clinical Center of Vojvodina, Novi Sad, Serbia
Marko Dimitrijević General Hospital Zaječar, Zaječar, Serbia
Ružica Janković Tomašević University Clinical Center Niš, Cardiology Clinic, Niš, Serbia
Sonja Dakić University Clinical Center Niš, Cardiology Clinic, Niš, Serbia; University of Niš, Faculty of Medicine, Niš, Serbia
Nenad Božinović University Clinical Center Niš, Cardiology Clinic, Niš, Serbia; University of Niš, Faculty of Medicine, Niš, Serbia
Milena Pavićević University Clinical Center Niš, Cardiology Clinic, Niš, Serbia

DOI: https://doi.org/10.2298/VSP230218026D

Keywords: diagnosis, histological techniques, myocarditis, eosinophilia, churg-strauss syndrome

Abstract

Introduction. Myocarditis is not a rare diagnosis, but its etiology often remains unknown as it requires extensive diagnostic work. Eosinophilic granulomatosis with polyangiitis (EGPA) or Churg-Strauss syndrome is a very rare systemic disease that is not easy to diagnose. Myocarditis in EGPA is uncommon and usually occurs in the late stages of the disease. Case report. A 22-year-old man was admitted with acute coronary syndrome. Using coronary angiography, the presence of stenoses on the epicardial coronary arteries was ruled out, and a working diagnosis of myocardial infarction with non-obstructive coronary arteries (MINOCA) was established. Then, we found inflammatory syndrome, eosinophilia, and a lot of systemic symptoms and signs. The diagnostic work included extensive laboratory tests, which ruled out infectious agents. Then, immunological tests, a computed tomography scan of the chest, cardiac magnetic resonance imaging (MRI) and a biopsy of the bone marrow, nasal mucosa, and skin were performed. We managed to establish the diagnosis of myopericarditis by cardiac MRI. The cause of myocarditis – EGPA, was found only after the histopathological finding of the skin biopsy, which enabled adequate immunosuppressive therapy. Conclusion. The accurate diagnosis was crucial for the correct, causal treatment of the patient, especially because he needed life-long immunosuppressive therapy. In order for such complex patients to receive adequate treatment, a multidisciplinary approach and perseverance in the diagnostic evaluation of the etiology of myocarditis are necessary.

Author Biography

Miodrag Damjanović, University Clinical Center Niš, Cardiology Clinic, Niš, Serbia

References

Churg J, Strauss L. Allergic granulomatosis, allergic angiitis, and periarteritis nodosa. Am J Pathol 1951; 27(2): 277–301.

Jennette JC, Falk RJ, Andrassy K, Bacon PA, Churg J, Gross WL, et al. Nomenclature of systemic vasculitides. Proposal of an international consensus conference. Arthritis Rheum 1994; 37(2): 187–92.

Masi AT, Hunder GG, Lie JT, Michel BA, Bloch DA, Arend WP, et al. The American College of Rheumatology 1990 criteria for the classification of Churg-Strauss syndrome (allergic granulomatosis and angiitis). Arthritis Rheum 1990; 33(8): 1094–100.

Comarmond C, Pagnoux C, Khellaf M, Cordier JF, Hamidou M, Viallard JF, et al. Eosinophilic granulomatosis with polyangiitis (Churg-Strauss): clinical characteristics and long-term followup of the 383 patients enrolled in the French Vasculitis Study Group cohort. Arthritis Rheum 2013; 65(1): 270–81.

Lopes PM, Rocha BML, Cunha GJL, Ranchordas S, Albuquerque C, Ferreira AM, et al. Fulminant Eosinophilic Myocarditis: A rare and life-threatening presentation of eosinophilic granulomatosis with polyangiitis. JACC Case Rep 2020; 2(5): 802–8.

Qiao L, Gao D. A case report and literature review of Churg–Strauss syndrome presenting with myocarditis. Medicine (Bal-timore) 2016; 95(51): e5080.

Mahr A, Moosig F, Neumann T, Szczeklik W, Taillé C, Vaglio A, et al. Eosinophilic granulomatosis with polyangiitis (Churg-Strauss): evolutions in classification, etiopathogenesis, assess-ment and management. Curr Opin Rheumatol 2014; 26(1): 16–23.

Hazebroek MR, Kemna MJ, Schalla S, Sanders-van Wijk S, Gerretsen SC, Dennert R, et al. Prevalence and prognostic relevance of cardiac involvement in ANCA-associated vasculitis: eosinophilic granulomatosis with polyangiitis and granulomatosis with polyangiitis. Int J Cardiol 2015; 199: 170–9.

Nanzer AM, Wechsler ME. Eosinophilic granulomatosis with polyangiitis. In: Jackson DJ, Wechsler ME, editors. Eosinophilic Lung Diseases. Sheffield: European Respiratory Society; 2022. p. 177–92. (ERS Monograph)

Wechsler ME, Akuthota P, Jayne D, Khoury P, Klion A, Langford CA, et al. Mepolizumab or placebo for eosinophilic granulo-matosis with polyangiitis. N Engl J Med 2017; 376(20): 1921–32.

Grayson PC, Ponte C, Suppiah R, Robson JC, Craven A, Judge A, et al. 2022 American College of Rheumatology/European Alliance of Associations for Rheumatology Classification Criteria for Eosinophilic Granulomatosis With Polyangiitis. Arthritis Rheumatol 2022; 74(3): 386‒92.

Moosig F, Bremer JP, Hellmich B, Holle JU, Holl-Ulrich K, Laudien M, et al. A vasculitis centre based management strategy leads to improved outcome in eosinophilic granulomatosis and polyangiitis (Churg-Strauss, EGPA): monocentric experiences in 150 patients. Ann Rheum Dis 2013; 72(6): 1011–7.

Neumann T, Manger B, Schmid M, Kroegel C, Hansch A, Kaiser WA, et al. Cardiac involvement in Churg-Strauss syndrome: impact of endomyocarditis. Medicine (Baltimore) 2009; 88(4): 236‒43.

Bluett R, McDonnell D, O’Dowling C, Vaughan C. Eosinophilic myocarditis as a first presentation of eosinophilic granulo-matosis with polyangiitis (Churg-Strauss syndrome). BMJ Case Rep 2017; 2017: bcr2017221227.

Caforio AL, Pankuweit S, Arbustini E, Basso C, Gimeno-Blanes J, Felix SB, et al. Current state of knowledge on aetiology, diag-nosis, management, and therapy of myocarditis: a position statement of the European Society of Cardiology Working Group on Myocardial and Pericardial Diseases. Eur Heart J 2013; 34(33): 2636–48; 2648a–d.

Sinico RA, Di Toma L, Maggiore U, Bottero P, Radice A, Tosoni C, et al. Prevalence and clinical significance of antineutrophil cy-toplasmic antibodies in Churg-Strauss syndrome. Arthritis Rheum 2005; 52(9): 2926–35.

Khoury P, Grayson PC, Klion AD. Eosinophils in vasculitis: characteristics and roles in pathogenesis. Nat Rev Rheumatol 2014; 10(8): 474–83.

Załęska J, Wiatr E, Zych J, Szopiński J, Oniszh K, Kober J, et al. Severe congestive heart failure as the main symptom of eosin-ophilic granulomatosis and polyangiitis (Churg-Strauss syn-drome). Pneumonol Alergol Pol 2014; 82(6): 582–9.

Bourgarit A, Toumelin PL, Pagnoux C, Cohen P, Mahr A, Guern VL, et al. Deaths occurring during the first year after treat-ment onset for polyarteritis nodosa, microscopic polyangiitis, and Churg-Strauss syndrome: a retrospective analysis of causes and factors predictive of mortality based on 595 patients. Medicine (Baltimore) 2005; 84(5): 323‒30.

Caforio ALP, Adler Y, Agostini C, Allanore Y, Anastasakis A, Arad M, et al. Diagnosis and management of myocardial in-volvement in systemic immune-mediated diseases: a position statement of the European Society of Cardiology Working Group on Myocardial and Pericardial Disease. Eur Heart J 2017; 38(35): 2649‒62.

d‘Ersu E, Ribi C, Monney P, Vincenti G, Schwitter J, Rotman S, et al. Churg-Strauss syndrome with cardiac involvement: case il-lustration and contribution of CMR in the diagnosis and clini-cal follow-up. Int J Cardiol 2018; 258: 321‒4.