Journal
CURRENT OPINION IN RHEUMATOLOGY
Volume 22, Issue 6, Pages 696-703Publisher
LIPPINCOTT WILLIAMS & WILKINS
DOI: 10.1097/BOR.0b013e32833dfbd8
Keywords
pulmonary arterial hypertension; scleroderma; scleroderma heart disease; systemic sclerosis
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Funding
- NIH/NHLBI [P50 HL084946, K23 HL092287]
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Purpose of review Systemic sclerosis (SSc), a collagen vascular disease characterized by diverse organ system involvement, is associated with serious cardiac complications. Cardiac symptoms are much less frequent than autopsy-demonstrated cardiac involvement. Although frequent calls for early diagnosis have been made, validated strategies for assessment of scleroderma cardiac disease are not yet well established, mainly because the right ventricle, a common target of the disease, presents considerable obstacles to simple and reliable appraisal of its structure and function. This article reviews the current information about cardiac dysfunction in scleroderma, with special emphasis on its detection and prognostic implications. Recent findings Cardiac involvement is a harbinger of poor prognosis in patients with SSc. Recent progress in its diagnosis has been made with the availability of more sophisticated diagnostic tools. Summary Early detection of scleroderma heart disease will allow exploration of novel therapies with potential positive impact on the quality of life and life expectancy of this patient population.
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