Review
Clinical cardiac involvement in idiopathic inflammatory myopathies: A systematic review

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Abstract

Background

Idiopathic inflammatory myopathies (IIM) presenting with diffuse skeletal muscular inflammation and cardiac involvement is one of the major causes of clinical deterioration. Our purpose was to observe the frequency of various reported clinical cardiac afflictions, cardiovascular mortality and its most important contributors in the IIM population.

Methods

MEDLINE database was searched from 1977 through 2009. Articles reporting objective evidence of cardiac involvement were assessed. Patients were included if they satisfied Bohan and Peter criteria for definite or probable diagnosis of IIM. The review conforms to the criteria of the new PRISMA statement of preferred reporting items for systematic reviews and meta-analyses.

Results

Thirty-three out of 90 articles reported cardiac data. Twelve were case reports, while 21 articles were either cohort studies or case series. Angina was reported in 7% of cumulative prospective cohort. Dysrhythmias were seen in 31.8% of electrocardiograms. Diastolic dysfunction was the most common echocardiographic finding. Congestive heart failure was reported in 5.6% of the prospective cohort and was the most common cause of death accounting for 21% of total cardiac mortality. Myocarditis was the most common feature in reported pathology literature (38%), followed by focal myocardial fibrosis (22%).

Conclusion

Cardiac involvement is an important cause of morbidity and mortality in patients with IIM. Myocardial fibrosis puts the patient at risk for systolic or diastolic heart failure and dysrhythmias. This review, therefore, suggests the importance of early and comprehensive cardiac evaluation in IIM population. Confirmation of these findings will require prospective studies of consecutive patients with long-term follow-up.

Introduction

Myocardial damage secondary to the idiopathic inflammatory myopathy (IIM comprised of polymyositis (PM), dermatomyositis (DM) and inclusion body myositis) although documented in the literature, still carries a sense of controversy around it. Since the initial case report in 1899 by Oppenheim [1], there has been a general impression of heart involvement being a rare entity [2], [3]. As more data become available on this uncommon auto-immune disease, cardiovascular problems started appearing as the principal cause of death in patients with IIM [4]. The cardiac manifestations of IIM have been primarily described as being occult, mostly manifesting as conduction abnormalities in case reports. There have been reports of clinically evident congestive heart failure (CHF) and heart block. Whether this reflects the muscle inflammation or is secondary to coronary or small vessel vasculitis is still controversial.

Poor prognostic factors for IIM patients have been described as having an associated neoplasm, age over 45 and the presence of coexisting heart or lung involvement [5], [6], [7]. One study concluded that cardiovascular manifestations were the most common cause of death in IIM patients [8]. The controversy over the prevalence of heart involvement in IIM may also be due to the advent of highly sensitive diagnostic techniques, as it has now become possible to diagnose subclinical heart involvement in these disorders and hence the frequency of detecting heart abnormalities in such patients has increased [2], [3]. With a lack of comprehensive epidemiological data, the actual frequency of cardiac morbidity and mortality in IIM still remains to be determined.

To our knowledge, there has never been a systematic review of the significance of cardiovascular involvement in patients with IIM and hence this review was motivated by the following concerns: 1) What is the frequency of clinical cardiac abnormalities in patients with IIM (using sensitive methods to detect conduction abnormality or left ventricular dysfunction). 2) Is cardiac involvement a major cause of mortality in patients with IIM. 3) What are the various causes of cardiac mortality in IIM patients. 4) Does IIM treatment lead to recovery of cardiac function.

Section snippets

Data sources and search strategy

Ovid software was used to search the MEDLINE database from January 1977 through December 2009. Articles containing the keywords relating to IIM (polymyositis, dermatomyositis, myositis, idiopathic inflammatory myopathies) as concerns to heart conditions (angina, congestive heart failure, myocardial infarction, heart bock, conduction defects, ECG changes, troponin, creatinine kinase-MB, echocardiogram, and chest pain) were used. Articles, case reports, prospective and retrospective studies that

Article and patient characteristics

Thirty-three articles out of 90 were selected according to the pre-specified selection criteria. Twelve of these were case reports contributing 14 patients [12], [13], [14], [15], [16], [17], [18], [19], [20], [21], [22], [23]. The other 21 articles were either cohort studies or case series. There were 11 prospective series, 8 retrospective chart reviews and 2 retrospective autopsy reviews. Table 2 documents the characteristics of patients in case reports [12], [23] and Table 3 depicts the same

Discussion

Although anecdotal evidence has been suggesting the occurrence of cardiac involvement in IIM for over 50 years, detailed objective evidence for clinical cardiac involvement is surprisingly limited. Our search identified only 33 articles with cardiac data. Of these, 10 of them described one patient each while 2 had two patients each. Eighteen cohort studies were identified, 11 of which reported prospective data and 10 were retrospective chart reviews. These observations encompass a broad range

Acknowledgement

The authors of this manuscript have certified that they comply with the Principles of Ethical Publishing in the International Journal of Cardiology [49].

Disclosures: authors have nothing to disclose and no conflict of interest.

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