Scleroderma (cardiac manifestations)

Last revised by Daniel J Bell on 30 Mar 2021

Citation, DOI, disclosures and article data

Citation:

Hacking C, Bell D, Botz B, Scleroderma (cardiac manifestations). Reference article, Radiopaedia.org (Accessed on 20 Apr 2024) https://doi.org/10.53347/rID-74254

DOI:

https://doi.org/10.53347/rID-74254

Permalink:

https://radiopaedia.org/articles/74254

rID:

74254

Article created:

12 Feb 2020, Craig Hacking ◉ ◈

Disclosures:

At the time the article was created Craig Hacking had no recorded disclosures.

View Craig Hacking's current disclosures

Last revised:

30 Mar 2021, Daniel J Bell ◉

Disclosures:

At the time the article was last revised Daniel J Bell had no recorded disclosures.

View Daniel J Bell's current disclosures

Revisions:

4 times, by 3 contributors - see full revision history and disclosures

Systems:

Cardiac

Synonyms:

Cardiac manifestations of scleroderma

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.

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
- 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 (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)