Scleroderma (cardiac manifestations)

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.

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
• 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
• pericardial disease, which is commonly asymptomatic
  • fibrinous pericarditis
  • fibrous pericarditis
  • pericardial adhesions
  • pericardial effusion
  • pericardial tamponade (rare)
  • constrictive pericarditis (rare)
• valvular vegetations (rare)

Secondary cardiac involvement

• pulmonary arterial hypertension (approximately 10% ¹)
• interstitial lung disease
• renal disease

Radiographic features

Cardiac MRI

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 (c.f. with atherosclerosis in which it is spared)
  • typically the mid-wall regions
• contraction band necrosis with no particular distribution
• concentric intimal hypertrophy
• absence of haemosiderin deposition (c.f. with atherosclerosis in which it is typical)

See also

• pulmonary manifestations of scleroderma
• gastrointestinal manifestations of scleroderma
• hepatobiliary manifestations of scleroderma
• renal manifestations of scleroderma
• differential diagnosis of erosive arthritis