Synopsis
Cardiac
manifestations are frequently observed in many inflammatory systemic disorders.
Identification of cardiac involvement is of high clinical importance, as in
many instances a large proportion of the morbidity and mortality in systemic
inflammatory diseases is due to cardiac complications, which if identified
early may be amenable to therapeutic intervention. In order to simplify the
protean cardiac manifestations observed in inflammatory systemic disorders,
these diseases can be discussed under the general headings of sarcoidosis,
connective tissue diseases, and hypereosinophilic syndromes.Target Audience
Cardiac
imaging physicians, particularly those involved with cardiac magnetic resonance
(CMR) imaging
Outcome/Objectives
To
develop a greater understanding of the role CMR can play in the evaluation of
cardiac pathology in patients with inflammatory systemic disorders
Cardiac manifestations
of inflammatory systemic disorders
Sarcoidosis
Sarcoidosis is a multisystem
granulomatous disease characterised by non-caseating granulomata in affected
organs. Cardiac involvement in
sarcoidosis is present 20-30% of
patients at post mortem studies in the US, and can complicate up to 60% of
sarcoidosis cases in Japan, where it is responsible for up to 80% of deaths.
Common clinical consequences are conduction disturbances, ventricular
arrhythmias and heart failure.
Whilst
there is no gold standard for the diagnosis of cardiac involvement in sarcoidosis,
CMR imaging has greatly enhanced the detection of cardiac involvement. The
principle features detected on CMR are cardiac inflammation, and also cardiac
scarring, which can also be used to guide specific therapies, such as
immunosuppression or device therapy.
Connective tissue
diseases
Considered
within this group are a range of overlapping clinical syndromes, including
systemic lupus erythematosus (SLE), rheumatoid arthritis, inflammatory myositis
and scleroderma. Cardiac inflammation has been observed in all these conditions
with varying frequency; however the detection of myocardial inflammation with
CMR has enabled earlier detection of cardiac changes that are often
subclinical. In some instances, such as in patients with SLE, the rate of subclinical
cardiac involvement may be significantly higher than that previously thought
prior to the advent of CMR imaging, suggesting a potential role for early
therapeutic intervention. However to date there are very limited data testing therapeutic
interventions in any of these syndromes.
Hypereosinophilic
syndromes
Hypereosinophilia
can occur as a consequence of a broad range of aetiologies, including allergic
and autoimmune, infective and haematological disorders, as well as in specific
syndromes such as Churg-Strauss Syndrome. Characteristic features of cardiac
inflammation in hypereosinophilic syndromes are endomyocardial fibrosis,
particularly involving the ventricular apices, and ventricular thrombus
formation, both of which are well evaluated with CMR imaging. In many instances
patients achieve a very good response to immunosuppressive therapy.
Conclusion
Whilst
a broad range of cardiac manifestations may occur in inflammatory systemic
disorders, the recent application of CMR, particularly with respect to
identifying myocardial inflammation, scar and cardiac thrombosis represent
major advances in the early detection and therapeutic intervention in these
diseases.
Acknowledgements
No acknowledgement found.References
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