Scleroderma (cardiac manifestations)

Last revised by Daniel J Bell on 30 Mar 2021

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.

Patients may present with:

  • myocardial infarction
  • diastolic dysfunction
  • palpitations and arrhythmias

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.

Numerous features are found in myocardial disease which includes 1,3,4 :

  • 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)

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