The heart in progressive systemic sclerosis (scleroderma)

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The heart in progressive systemic sclerosis (scleroderma)
Scleroderma heart 1.jpg
This is a gross photograph of the heart from this case. There is thickening of the left ventricular wall and some thickening of the right ventricle as well.
ICD-10 M34.
ICD-9 710.1
OMIM 181750
DiseasesDB 12845
MedlinePlus 000429
MeSH D012595

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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] Assistant Editor-In-Chief: Brian Blank

Overview

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

  • conduction system defects
  • valvular abnormalities

It's generally thought cardiac disease is a major cause of death from scleroderma, or that it often contributes to death, though cardiac disease is only reported in about 10 percent of cases. Conduction abnormalities, myocardial and pericardial disease disease may also be common. Some have suggested a 'cardiac Raynaud's phenomenon' exists, but exactly how myocardial involvement develops in scleroderma is unclear.

An angiography-based study of 172 patients has suggested patients who may have obstructive epicardial disease associated 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. 1.0 1.1 Akram MR, Handler CE, Williams M, et al (November 2006). "Angiographically proven coronary artery disease in scleroderma". Rheumatology (Oxford) 45 (11): 1395–8. doi:10.1093/rheumatology/kel120. PMID 16606654.



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