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• Myocarditis in poly- /dermatomyositis on cardiac MRI before and after treatment
  josef finsterer
  Published on: 25 April 2006

• Published on: 25 April 2006

  Myocarditis in poly- /dermatomyositis on cardiac MRI before and after treatment

  • josef finsterer, neurologist
  • Other Contributors:
    • Claudia Stöllberger

  Dear Editor,

  With interest we read the article by Allanore et al. on three patients with polymyositis (PM) and one with dermatomyositis (DM) in whom myocarditis was diagnosed and the therapeutic effect of immunosuppressants monitored by contrast enhanced cardiac MRI (cMRI) [1]. The report raises the following questions and concerns: Cardiac involvement in DM/PM has been repeatedly described and usually remains asy...

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  Dear Editor,

  With interest we read the article by Allanore et al. on three patients with polymyositis (PM) and one with dermatomyositis (DM) in whom myocarditis was diagnosed and the therapeutic effect of immunosuppressants monitored by contrast enhanced cardiac MRI (cMRI) [1]. The report raises the following questions and concerns: Cardiac involvement in DM/PM has been repeatedly described and usually remains asymptomatic [2,3,4]. The most frequent symptomatic manifestations are arrhythmias, myocarditis, or arteritis [3,5,6,7]. Rare cardiac manifestations are heart failure [2], pericarditis [4], pericardial tamponade [6], restrictive cardiomyopathy [8], or pulmonary hypertension [3].

  Did the authors observe cardiac manifestations other than myocarditis in their four patients? Was ST-segment elevation and septal hypokinesia in patient 2 and mitral insufficiency in patient 3 attributable to DM/PM? Diagnosis of myocarditis is a challenge and relies on the determination of CK, CK-MB, gallium citrate or indium labeled anti-myosin antibodies, ECG, AECG, echocardiography, and myocardial biopsy. A further possible tool may be myocardial scintigraphy [9]. Which is the evidence that the described abnormalities indeed represent myocarditis due to DM/PM? The references given only refer to viral myocarditis but not to DM/PM. Was the cMRI diagnosis confirmed by any of the conventional diagnostic techniques? Were CK-MB values elevated in any of the 4 patients? Was coronary angiography carried out in any patient to confirm possible vasculitis? Was endomyocardial biopsy carried out in any of the four patients?

  How to explain that all four patients had evidence of myocarditis although myocarditis can be found in only 30% of the DM/PM patients at autopsy [10]? According to the results, two patients reported cardiac symptoms. In table 1, however, all four patients are mentioned to have had cardiac symptoms. It has been also reported that the cardiac BMIPP SPECT may be positive in myocarditis in DM/PM [11].

  Regions with reduced tracer uptake relate to wall motion abnormalities. Were such investigations carried out in any of the four patients to confirm the cMRI findings? How to explain that patient four, who had the largest myocardial area affected in the anteroseptal wall on cMRI did not show any abnormality on echocardiography or ECG. Did he have elevated CK-MB or troponin T levels? Did all patients undergo muscle biopsy, to which degree were patients affected neurologically, and were the neurological abnormalities related tot the cardiac abnormalities? DM/PM is frequently associated with malignancy [12].

  Was DM/PM in any of the four patients a paraneoplastic phenomenon? Regarding figure 1 it seems that both images represent different image planes and are thus not comparable with regard to the therapeutic effect of corticosteroids. Although contrast cMRI may have a potential to support the diagnosis of myocarditis in DM/PM this technique has to be compared with a golden standard for diagnosing myocarditis, like endomyocardial biopsy, before finally assessing its value in the diagnosis of myocarditis in DM/PM. As long as the sensitivity and specificity remains unknown, cMRI can not be recommended as a tool for diagnosing or or monitoring treatment of myocarditis in DM/PM.

  References

  1 Allanore Y, Vignaux O, Arnaud L, Puechal X, Pavy S, Duboc D, Legmann P, Kahan A. Effects of corticosteroids and immunosuppressors on idiopathic inflammatory myopathy related myocarditis evaluated by magnetic resonance imaging. Ann Rheum Dis 2006;65:249-52.

  2 Cuny C, Eicher JC, Collet E, Chatard C, Chauffert B, Lorcerie B, Martin F, Wolf JE, Louis P. Dilated cardiomyopathy disclosing dermatopolymyositis. Management. Ann Cardiol Angeiol (Paris) 1993;42:155- 8.

  3 Lundberg IE. Cardiac involvement in autoimmune myositis and mixed connective tissue disease. Lupus 2005;14:708-12.

  4 Tami LF, Bhasin S. Polymorphism of the cardiac manifestations in dermatomyositis. Clin Cardiol 1993;16:260-4.

  5 Alyan O, Ozdemir O, Geyik B, Demirkan D. Polymyositis complicated with complete atrioventricular block - a case report and review of the literature. Angiology 2003;54:729-31.

  6 Pereira RM, Lerner S, Maeda WT, Goldenstein-Schainberg C, Cossermelli W. Pericardial tamponade in juvenile dermatomyositis. Clin Cardiol 1992;15:301-3.

  7 Vinsonneau U, Delluc A, Bergez C, Caumes D, Talarmin F. Second degree atrioventricular block in mixed connective tissue disease. Rev Med Interne 2005;26:656-60.

  8 Finsterer J, Stöllberger C, Avanzini M, Rauschka H: Restrictive cardiomyopathy in dermatomyositis. Scand J Rheumatol 2006;(in press)

  9 Buchpiguel CA, Roizemblatt S, Pastor EH, Hironaka FH, Cossermelli W. Cardiac and skeletal muscle scintigraphy in dermato- and polymyositis: clinical implications. Eur J Nucl Med 1996;23:199-203.

  10 Lie JT. Cardiac manifestations in polymyositis/dermatomyositis: how to get to the heart of the matter? J Rheumatol 1995;22:809-11.

  11 Ito K, Sugihara H, Zen K, Hikosaka T, Adachi Y, Tanabe T, Yoneyama S, Katoh S, Nakamura T, Azuma A. Clinical usefulness of 123I-BMIPP myocardial SPECT in collagen disease. Kaku Igaku 2000;37:327-32.

  12 Dalakas MC, Hohlfeld R. Polymyositis and dermatomyositis. Lancet 2003;362:971-82.

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  Conflict of Interest:
  None declared.

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