The heart in progressive systemic sclerosis (scleroderma)

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Associate Editor-In-Chief: Cafer Zorkun, M.D., Ph.D. [2]

Please Join in Editing This Page and Apply to be an Editor-In-Chief for this topic: There can be one or more than one Editor-In-Chief. You may also apply to be an Associate Editor-In-Chief of one of the subtopics below. Please mail us [3] to indicate your interest in serving either as an Editor-In-Chief of the entire topic or as an Associate Editor-In-Chief for a subtopic. Please be sure to attach your CV and or biographical sketch.

Overview

Severe complications from Progressive Systemic Scleroderma (Scleroderma) can include:

  • Untreated high blood pressure that strains the heart
  • irregular heart rhythm
  • enlargement of the heart, which leads to heart failure.

It's generally thought cardiac disease is a major cause of death from scleroderma, or that it often contributes to death. Conduction abnormalities, myocardial and pericardial disease disease may also be common.

An angiography-based study of 172 patients has suggested patients who may have obstructive epicardial disease associate with scleroderma should be treated the same as patients without scleroderma.[1]

Pathology

Images courtesy of Professor Peter Anderson DVM PhD and published with permission © PEIR, University of Alabama at Birmingham, Department of Pathology

This is a gross photograph of the heart from a patient with scleroderma. There is thickening of the left ventricular wall and some thickening of the right ventricle as well.


Scleroderma: Right ventricular hypertrophy due to pulmonary fibrosis in Scleroderma


Scleroderma: Right ventricular hypertrophy due to pulmonary fibrosis in Scleroderma


Scleroderma: Right ventricular hypertrophy due to pulmonary fibrosis in Scleroderma


References

  1. Akram MR, Handler CE, Williams M; et al. (2006). "Angiographically proven coronary artery disease in scleroderma". Rheumatology (Oxford). 45 (11): 1395–8. doi:10.1093/rheumatology/kel120. PMID 16606654. Unknown parameter |month= ignored (help)


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