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Review
. 2024 Jul 1;63(7):1825-1836.
doi: 10.1093/rheumatology/keae029.

Advances in the diagnosis of myocarditis in idiopathic inflammatory myopathies: an overview of diagnostic tests

Gautam Sen  1   2   3 , Paul Scully  4 , Patrick Gordon  5 , Daniel Sado  1   3
Affiliations
Review

Advances in the diagnosis of myocarditis in idiopathic inflammatory myopathies: an overview of diagnostic tests

Gautam Sen et al. Rheumatology (Oxford). .

Abstract

Cardiac involvement in idiopathic inflammatory myopathies (IIM) purports to worse clinical outcomes, and therefore early identification is important. Research has focused on blood biomarkers and basic investigations such as ECG and echocardiography, which have the advantage of wide availability and low cost but are limited in their sensitivity and specificity. Imaging the myocardium to directly look for inflammation and scarring has therefore been explored, with a number of new methods for doing this gaining wider research interest and clinical availability. Cardiovascular magnetic resonance (CMR) with contemporary multiparametric mapping techniques and late gadolinium enhancement imaging, is an extremely valuable and increasingly used non-invasive imaging modality for the diagnosis of myocarditis. The recently updated CMR-based Lake Louise Criteria for the diagnosis of myocarditis incorporate the newer T1 and T2 mapping techniques, which have greatly improved the diagnostic accuracy for IIM myocarditis.18F-FDG-PET/CT is a well-utilized imaging modality in the diagnosis of malignancies in IIM, and it also has a role for the diagnosis of myocarditis in multiple systemic inflammatory diseases. Endomyocardial biopsy, however, remains the gold standard technique for the diagnosis of myocarditis and is necessary for the diagnosis of specific cases of myocarditis. This article provides an overview of the important tests and imaging modalities that clinicians should consider when faced with an IIM patient with potential myocarditis.

Keywords: 18F-FDG-PET/CT; cardiac biomarkers; cardiac magnetic resonance; creatine kinase; endomyocardial biopsy; idiopathic inflammatory myopathies; troponin I.

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Figures

None
Graphical abstract
Figure 1.
Figure 1.
Cardiac magnetic resonance late gadolinium enhancement patterns showing ischaemic and non-ischaemic pathologies
Figure 2.
Figure 2.
Cardiac magnetic resonance late gadolinium enhancement pattern showing normal myocardium compared with myocarditis
Figure 3.
Figure 3.
Cardiac magnetic resonance T1 mapping. (A and B) Long axis 3-chamber and short axis views showing normal T1 values within the myocardium. (C and D) Long axis 3-chamber and short axis views showing elevated T1 values at the basal lateral wall (peak T1 value of 1300 ms, with normal <1100 ms) (white arrows) in a patient with SSc, consequently diagnosed with myocarditis. The high signal at the lateral wall within the myocardium shows high T1 signal compared with the rest of the myocardium
Figure 4.
Figure 4.
Cardiac magnetic resonance T2 mapping. (A and B) Long axis 3-chamber and short axis views showing normal T2 values within the myocardium. (C and D) Long axis 3-chamber and short axis view of a patient with SLE consequently diagnosed with myocarditis. There is diffuse T2 elevation in the whole myocardium, with a peak seen at the basal inferior/infero-lateral walls (black arrowheads), with an elevated T2 value of 65 ms (normal value <48ms). Elevation of T2 is a marker of acute myocardial inflammation and oedema, often seen in acute myocarditis. There is also a large pericardial effusion (black star)
Figure 5.
Figure 5.
Cardiac magnetic resonance in an IIM patient. (A and B) Short axis and long axis 3-chamber T2 mapping images showing elevated myocardial T2 values in the basal lateral wall (black arrows). (C and D) Short axis and long axis 3-chamber late gadolinium images showing mid wall late enhancement in the basal lateral wall (white arrows). Elevation of T2 is a marker of acute myocardial inflammation, often seen in acute myocarditis. Late enhancement in the mid wall is a classic finding in myocarditis
Figure 6.
Figure 6.
18-FDG-PET/CT showing cardiac sarcoid. 18-FDG-PET/CT transverse images showing multiple intensely avid areas of FDG uptake (A) in mediastinal and hilar lymph nodes, (B) in the right ventricle and (C) in the left ventricle, with the anteroseptum showing the highest level of FDG uptake. A diagnosis of cardiac sarcoidosis was made. 18-FDG-PET/CT: fluorine-18 fluorodeoxyglucose PET/CT; FDG: fluorodeoxyglucose.
See this image and copyright information in PMC

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