Analysis of the clinical characteristics of cardiac injury associated with antimitochondrial antibodies_West China Medical Journal

Authors：

LI Ziqi , CUI Beibei , XIE Qibing ,  YIN Geng

Department of Rheumatology and Immunology, West China Hospital, Sichuan University, Chengdu, Sichuan 610041, P. R. China;

Corresponding author：

YIN Geng, Email: yingeng1975@163.com

Keywords：

Antimitochondrial antibodies; cardiac injury; clinical characteristics

DOI：

10.7507/1002-0179.202407331

Video：

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Objective To analyze the clinical characteristics of antimitochondrial antibody (AMA)-associated cardiac injury. Methods AMA positive patients admitted to West China Hospital of Sichuan University between June 2008 and November 2023 were retrospectively selected. They were categorized into the simple cardiac involvement group and the cardiac and skeletal muscle involvement group according to the presence of skeletal muscle injury. Differences in demographic characteristics, serologic indices, cardiac structure and function, and arrhythmias were compared between the two groups. Results A total of 55 AMA-M2 positive patients with myocardial injury were enrolled. There were 18 cases in the simple cardiac involvement group and 37 cases in the cardiac and skeletal muscle involvement group among them. The age (P=0.002) and mortality rate (P=0.031) of the simple cardiac involvement group were higher than those of the cardiac and skeletal muscle involvement group. There were significant differences in biochemical indicators such as lactate dehydrogenase and α-hydroxybutyrate dehydrogenase between the two groups, and the levels of myocardial enzymes such as creatine kinase isoenzyme, myoglobin and troponin T in the cardiac involvement group were lower than those in the cardiac with skeletal muscle involvement group (P<0.05). Meanwhile, there were significant differences in the incidence of atrial premature beats, atrial fibrillation and other arrhythmias between the two groups (P<0.05). In terms of treatment modalities, glucocorticoids and immunosuppressants were used more frequently in the cardiac combined skeletal muscle involvement group than in the cardiac involvement alone group, whereas β-blockers and diuretics were more prevalent in the cardiac involvement alone group (P<0.05). Conclusion Patients with cardiac involvement alone may have a more insidious or rapid progression of the disease, which requires clinicians to pay higher attention to and provide timely and effective treatment, thus improving the overall prognosis and quality of life of the patients.

Citation： LI Ziqi, CUI Beibei, XIE Qibing, YIN Geng. Analysis of the clinical characteristics of cardiac injury associated with antimitochondrial antibodies. West China Medical Journal, 2025, 40(2): 269-275. doi: 10.7507/1002-0179.202407331 Copy

1.	MACKAY IR. Primary biliary cirrhosis showing a high titer of autoantibody; report of a case. N Engl J Med, 1958, 258(4): 185-188.
2.	GAJDUSEK DC. An autoimmune reaction against human tissue antigens in certain acute and chronic diseases. I. Serological investigations. AMA Arch Intern Med, 1958, 101(1): 9-29.
3.	WALKER JG, DONIACH D, ROITT IM, et al. Serological tests in diagnosis of primary biliary cirrhosis. Lancet, 1965, 1(7390): 827-831.
4.	Tuaillon N, Andre C, Briand JP, et al. A lipoyl synthetic octadecapeptide of dihydrolipoamide acetyltransferase specifically recognized by anti-M2 autoantibodies in primary biliary cirrhosis. J Immunol, 1992, 148(2): 445-450.
5.	Berg PA, Klein R, Lindenborn-Fotinos J. Antimitochondrial antibodies in primary biliary cirrhosis. J Hepatol, 1986, 2(1): 123-131.
6.	Colapietro F, Lleo A, Generali E. Antimitochondrial antibodies: from bench to bedside. Clin Rev Allergy Immunol, 2022, 63(2): 166-177.
7.	Dahlqvist G, Gaouar F, Carrat F, et al. Large-scale characterization study of patients with antimitochondrial antibodies but nonestablished primary biliary cholangitis. Hepatology, 2017, 65(1): 152-163.
8.	Maeda MH, Tsuji S, Shimizu J. Inflammatory myopathies associated with anti-mitochondrial antibodies. Brain, 2012, 135 (Pt 6): 1767-1777.
9.	Kadosaka T, Tsujinaga S, Iwano H, et al. Cardiac involvement with anti-mitochondrial antibody-positive myositis mimicking cardiac sarcoidosis. ESC Heart Fail, 2020, 7(6): 4315-4319.
10.	Gonzalez-Chapa JA, Macêdo MB, Lood C. The emerging role of mitochondrial dysfunction in the pathogenesis of idiopathic inflammatory myopathies. Rambam Maimonides Med J, 2023, 14(2): e0006.
11.	Huang R, Zhang X, Han Z, et al. Refractory ventricular tachycardia and heart failure due to anti-mitochondrial antibody-positive inflammatory myopathy. BMC Cardiovasc Disord, 2023, 23(1): 57.
12.	Albayda J, Khan A, Casciola-Rosen L, et al. Inflammatory myopathy associated with anti-mitochondrial antibodies: a distinct phenotype with cardiac involvement. Semin Arthritis Rheum, 2018, 47(4): 552-556.
13.	Zhang L, Yang H, Lei J, et al. Muscle pathological features and extra-muscle involvement in idiopathic inflammatory myopathies with anti-mitochondrial antibody. Semin Arthritis Rheum, 2021, 51(4): 741-748.
14.	Højgaard P, Witting N, Rossing K, et al. Cardiac arrest in anti-mitochondrial antibody associated inflammatory myopathy. Oxf Med Case Reports, 2021, 2021(3): omaa150.
15.	Hasegawa Y, Izumi D, Kashimura T, et al. Life-threatening ventricular arrhythmia and left ventricular dysfunction associated with anti-mitochondrial antibody-positive myositis: a case report. Eur Heart J Case Rep, 2021, 5(12): ytab469.
16.	Bohan A, Peter JB. Polymyositis and dermatomyositis (first of two parts). N Engl J Med, 1975, 292(7): 344-347.
17.	Konishi H, Fukuzawa K, Mori S, et al. Anti-mitochondrial M2 antibodies enhance the risk of supraventricular arrhythmias in patients with elevated hepatobiliary enzyme levels. Intern Med, 2017, 56(14): 1771-1779.
18.	Uenaka T, Kowa H, Ohtsuka Y, et al. Less limb muscle involvement in myositis patients with anti-mitochondrial antibodies. Eur Neurol, 2017, 78(5/6): 290-295.
19.	Huang Y, Liu H, Wu C, et al. Ventricular arrhythmia predicts poor outcome in polymyositis/dermatomyositis with myocardial involvement. Rheumatology (Oxford), 2021, 60(8): 3809-3816.
20.	Yano T, Nagano N, Fujito T, et al. Aborted sudden cardiac death in anti-mitochondrial antibody-positive cardiomyopathy. Eur Heart J Cardiovasc Imaging, 2022: jeac245.
21.	Kitaura A, Yamamoto R, Tsukimoto S, et al. Nine-second cardiac arrest in a patient with anti-mitochondrial antibody-positive myopathy under general anesthesia. Cureus, 2023, 15(4): e37436.
22.	Uenaka T, Kowa H, Sekiguchi K, et al. Myositis with antimitochondrial antibodies diagnosed by rectus abdominis muscle biopsy. Muscle Nerve, 2013, 47(5): 766-768.
23.	Kurihara Y, Shishido T, Oku K, et al. Polymyositis associated with autoimmune hepatitis, primary biliary cirrhosis, and autoimmune thrombocytopenic purpura. Mod Rheumatol, 2011, 21(3): 325-329.
24.	Kazama S, Kondo T, Ito R, et al. Dilated cardiomyopathy with anti-mitochondrial M2 antibody: a case series. J Cardiol Cases, 2023, 28(1): 11-15.
25.	Sabbagh SE, Pinal-Fernandez I, Casal-Dominguez M, et al. Anti-mitochondrial autoantibodies are associated with cardiomyopathy, dysphagia, and features of more severe disease in adult-onset myositis. Clin Rheumatol, 2021, 40(10): 4095-4100.
26.	Zhang L, Zhu H, Yang P, et al. Myocardial involvement in idiopathic inflammatory myopathies: a multi-center cross-sectional study in the CRDC-MYO Registry. Clin Rheumatol, 2021, 40(11): 4597-4608.

1. MACKAY IR. Primary biliary cirrhosis showing a high titer of autoantibody; report of a case. N Engl J Med, 1958, 258(4): 185-188.
2. GAJDUSEK DC. An autoimmune reaction against human tissue antigens in certain acute and chronic diseases. I. Serological investigations. AMA Arch Intern Med, 1958, 101(1): 9-29.
3. WALKER JG, DONIACH D, ROITT IM, et al. Serological tests in diagnosis of primary biliary cirrhosis. Lancet, 1965, 1(7390): 827-831.
4. Tuaillon N, Andre C, Briand JP, et al. A lipoyl synthetic octadecapeptide of dihydrolipoamide acetyltransferase specifically recognized by anti-M2 autoantibodies in primary biliary cirrhosis. J Immunol, 1992, 148(2): 445-450.
5. Berg PA, Klein R, Lindenborn-Fotinos J. Antimitochondrial antibodies in primary biliary cirrhosis. J Hepatol, 1986, 2(1): 123-131.
6. Colapietro F, Lleo A, Generali E. Antimitochondrial antibodies: from bench to bedside. Clin Rev Allergy Immunol, 2022, 63(2): 166-177.
7. Dahlqvist G, Gaouar F, Carrat F, et al. Large-scale characterization study of patients with antimitochondrial antibodies but nonestablished primary biliary cholangitis. Hepatology, 2017, 65(1): 152-163.
8. Maeda MH, Tsuji S, Shimizu J. Inflammatory myopathies associated with anti-mitochondrial antibodies. Brain, 2012, 135 (Pt 6): 1767-1777.
9. Kadosaka T, Tsujinaga S, Iwano H, et al. Cardiac involvement with anti-mitochondrial antibody-positive myositis mimicking cardiac sarcoidosis. ESC Heart Fail, 2020, 7(6): 4315-4319.
10. Gonzalez-Chapa JA, Macêdo MB, Lood C. The emerging role of mitochondrial dysfunction in the pathogenesis of idiopathic inflammatory myopathies. Rambam Maimonides Med J, 2023, 14(2): e0006.
11. Huang R, Zhang X, Han Z, et al. Refractory ventricular tachycardia and heart failure due to anti-mitochondrial antibody-positive inflammatory myopathy. BMC Cardiovasc Disord, 2023, 23(1): 57.
12. Albayda J, Khan A, Casciola-Rosen L, et al. Inflammatory myopathy associated with anti-mitochondrial antibodies: a distinct phenotype with cardiac involvement. Semin Arthritis Rheum, 2018, 47(4): 552-556.
13. Zhang L, Yang H, Lei J, et al. Muscle pathological features and extra-muscle involvement in idiopathic inflammatory myopathies with anti-mitochondrial antibody. Semin Arthritis Rheum, 2021, 51(4): 741-748.
14. Højgaard P, Witting N, Rossing K, et al. Cardiac arrest in anti-mitochondrial antibody associated inflammatory myopathy. Oxf Med Case Reports, 2021, 2021(3): omaa150.
15. Hasegawa Y, Izumi D, Kashimura T, et al. Life-threatening ventricular arrhythmia and left ventricular dysfunction associated with anti-mitochondrial antibody-positive myositis: a case report. Eur Heart J Case Rep, 2021, 5(12): ytab469.
16. Bohan A, Peter JB. Polymyositis and dermatomyositis (first of two parts). N Engl J Med, 1975, 292(7): 344-347.
17. Konishi H, Fukuzawa K, Mori S, et al. Anti-mitochondrial M2 antibodies enhance the risk of supraventricular arrhythmias in patients with elevated hepatobiliary enzyme levels. Intern Med, 2017, 56(14): 1771-1779.
18. Uenaka T, Kowa H, Ohtsuka Y, et al. Less limb muscle involvement in myositis patients with anti-mitochondrial antibodies. Eur Neurol, 2017, 78(5/6): 290-295.
19. Huang Y, Liu H, Wu C, et al. Ventricular arrhythmia predicts poor outcome in polymyositis/dermatomyositis with myocardial involvement. Rheumatology (Oxford), 2021, 60(8): 3809-3816.
20. Yano T, Nagano N, Fujito T, et al. Aborted sudden cardiac death in anti-mitochondrial antibody-positive cardiomyopathy. Eur Heart J Cardiovasc Imaging, 2022: jeac245.
21. Kitaura A, Yamamoto R, Tsukimoto S, et al. Nine-second cardiac arrest in a patient with anti-mitochondrial antibody-positive myopathy under general anesthesia. Cureus, 2023, 15(4): e37436.
22. Uenaka T, Kowa H, Sekiguchi K, et al. Myositis with antimitochondrial antibodies diagnosed by rectus abdominis muscle biopsy. Muscle Nerve, 2013, 47(5): 766-768.
23. Kurihara Y, Shishido T, Oku K, et al. Polymyositis associated with autoimmune hepatitis, primary biliary cirrhosis, and autoimmune thrombocytopenic purpura. Mod Rheumatol, 2011, 21(3): 325-329.
24. Kazama S, Kondo T, Ito R, et al. Dilated cardiomyopathy with anti-mitochondrial M2 antibody: a case series. J Cardiol Cases, 2023, 28(1): 11-15.
25. Sabbagh SE, Pinal-Fernandez I, Casal-Dominguez M, et al. Anti-mitochondrial autoantibodies are associated with cardiomyopathy, dysphagia, and features of more severe disease in adult-onset myositis. Clin Rheumatol, 2021, 40(10): 4095-4100.
26. Zhang L, Zhu H, Yang P, et al. Myocardial involvement in idiopathic inflammatory myopathies: a multi-center cross-sectional study in the CRDC-MYO Registry. Clin Rheumatol, 2021, 40(11): 4597-4608.

West China Medical Journal

Analysis of the clinical characteristics of cardiac injury associated with antimitochondrial antibodies

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