Cardiac manifestations of scleroderma are highly variable, seen in approximately 15% of patients and are associated with a poor prognosis 2.
For a discussion of scleroderma in general, refer to parent article: scleroderma.
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Clinical presentation
Patients may present with:
myocardial infarction
diastolic dysfunction
palpitations and arrhythmias
Pathology
The pathogenesis of cardiac involvement can be divided into primary and secondary disease, but the separate mechanisms may co-exist.
Most cardiac manifestations are thought to be due to the same pathogenesis as other systemic disease processes in scleroderma, representing a combination of microvasculature alteration with collagen deposition by fibroblasts and immune disturbances, although these are not entirely understood at present.
Like most other systemic inflammatory diseases, atheroslerosis is accelerated in patients with scleroderma.
Primary cardiac involvement
myocarditis, which is the most common manifestation
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fibrosis of the conduction system
atrial and ventricular tachyarrhythmias are due to myocardial fibrosis
conduction defects and bradyarrhythmias are due to fibrosis of the conduction system itself
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pericardial disease, which is commonly asymptomatic
pericardial adhesions
pericardial tamponade (rare)
constrictive pericarditis (rare)
valvular vegetations (rare)
Secondary cardiac involvement
pulmonary arterial hypertension (approximately 10% 1)
Radiographic features
MRI
Numerous features are found in myocardial disease which includes 1,3,4 :
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patchy or mosaic myocardial fibrosis (pathognomonic feature)
unrelated to coronary epicardial artery distribution
involving the immediate subendocardium (cf. with atherosclerosis in which it is spared)
typically the mid-wall regions
contraction band necrosis with no particular distribution
concentric intimal hypertrophy
absence of hemosiderin deposition (cf. with atherosclerosis in which it is typical)