Rheumatology Advance Access originally published online on May 3, 2007
Rheumatology 2007 46(7):1208-1209; doi:10.1093/rheumatology/kem077
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LETTERS TO THE EDITOR |
Myocardial disease in systemic vasculitis and autoimmune disease detected by cardiovascular magnetic resonance
University of Birmingham – Department of Cardiovascular Medicine
Correspondence to: Nicola Edwards. E-mail: n.c.edwards{at}bham.ac.uk
SIR, Cardiovascular disease is a major cause of morbidity and mortality in patients with inflammatory autoimmune and vasculitic diseases such as systemic lupus erythematosus (SLE) and Wegener's granulomatosis (WG) [1, 2]. An increased prevalence of subclinical atherosclerotic disease on carotid ultrasound [3] and coronary Electron Beam Computed Tomography (EBCT) [4] has led to the suggestion that accelerated atherosclerotic disease may account for the high cardiovascular mortality and morbidity. In keeping with these observations, SPECT-myocardial perfusion imaging has identified abnormalities of perfusion in almost one-third of subjects with SLE but no history of coronary artery disease [5]. Non-atherosclerotic disease processes may also be important. Cardiovascular risk in systemic vasculitic disease is not well predicted by Framingham risk scores [6] and non-atheromatous cardiac abnormalities including valvular, myocardial and pericardial disease are frequently present on echocardiographic and post-mortem studies [7–9]. This suggests a more complex aetiology and a significant contribution from the alterations in immune function and inflammation.
We have performed contrast-enhanced cardiac magnetic resonance (CMR) imaging on 100 patients as part of a double blind randomized controlled trial investigating premature cardiovascular disease in early chronic kidney disease (CKD). All patients recruited into this study had no history of ischaemic heart disease and well controlled 24 hour ambulatory blood pressure (<130/80). The protocol was approved by South Birmingham Local Research Ethics Committee and patients gave written informed consent.
In participants with SLE and WG, we have identified a high prevalence of myocardial abnormalities. In 11 patients (7 females, 4 males, mean age 51 years) with quiescent SLE (7) or WG (4) there were five patients with late gadolinium enhancement (LGE) within the left ventricular (LV) myocardium. The appearance of enhancement was consistent but did not correspond to the pattern expected in ischaemic damage and was not specific to coronary artery territories. The LGE was mid-wall, with a diffuse distribution indicative of regional areas of fibrosis. One patient also had a confluent area of LGE in a sub-epicardial location typical of myocardial inflammation (Fig. 1). In all five patients, LV function, dimensions and mass were all within normal limits. In the remaining cohort of 89 patients with early CKD from other, non-vasculitic/autoimmune aetiologies, only two had evidence of LGE. One was sub-endocardial in distribution, typical of myocardial infarction. In the other case, a patient with focal segmental glomerulosclerosis (FSGS) had mid-wall LGE as seen in SLE and WG.
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To our knowledge, this is the first report of mid-wall LGE on CMR in systemic vasculitic and autoimmune disease. The pattern and distribution of scarring suggests fibrosis rather than infarction, which might have been expected in view of previous SPECT data. The superior spatial resolution of MRI allows detection of scarring and myocardial characterization on contiguous 7 mm slices, which is not possible with SPECT and may explain why this observation has not been reported previously by other imaging techniques. It is, however, consistent with reports of myocardial fibrosis based on post-mortem observations [7, 8].
These observations raise the possibility that myocardial damage in SLE and WG is due to a combination of subclinical inflammatory and immunological processes rather than ‘conventional’ coronary artery disease alone. The distribution of scarring supports post-mortem studies, which have shown patchy myocarditis and myocardial fibrosis in both SLE and WG [2, 7]. Acute presentation with myocarditis is rare and is thought to occur only in those with active disease, usually in association with pericardial change. Our findings suggest that subclinical myocarditis in these conditions may be much more common than previously thought and may contribute to the high incidence of clinical cardiovascular events.
Mid-wall LGE has been reported in other myocardial diseases including hypertrophic cardiomyopathy, dilated cardiomyopathy and amyloidosis. There is no evidence that any of our patients had these disease processes including the single patient with FSGS. Mid-wall LGE has recently been reported as present in a small proportion of patients with end-stage kidney disease [10]. The associated cardiovascular comorbidities of such patients make the significance of this finding unknown but an association with underlying inflammatory disease cannot be excluded.
In summary, while markers of atherosclerosis are well recognized in systemic vasculitis and autoimmune disease, we report the first case series of myocardial fibrosis using CMR in these conditions. This raises the possibility that immune mediated damage as well as atherosclerotic disease may contribute to myocardial injury in these patients.
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Acknowledgements |
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 Top Acknowledgements References |
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This work was supported by the British Heart Foundation.
The authors have declared no conflicts of interest.
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TopAcknowledgementsReferences |
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