Cardiac complications of systemic sclerosis

The majority of patients with SSc are believed to have subclinical primary cardiac involvement. Overt cardiac manifestations of SSc are associated with poor prognosis and can be difficult to manage. Primary myocardial disease, i.e. without systemic or pulmonary hypertension and without significant p...

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Bibliographic Details
Published in:Rheumatology (Oxford, England) England), 2009-06, Vol.48 (suppl-3), p.iii45-iii48
Main Authors: Kahan, A., Coghlan, G., McLaughlin, V.
Format: Article
Language:English
Subjects:
Acute Disease
Adult
Arrhythmias
Biological and medical sciences
Cardiology. Vascular system
Cardiomyopathy
Diseases of the osteoarticular system
Fibrosis
Heart
Heart - physiopathology
Heart Diseases - complications
Heart Diseases - drug therapy
Heart Diseases - physiopathology
Humans
Male
Medical sciences
Myocardial fibrosis
Myocarditis. Cardiomyopathies
Pericarditis
Sarcoidosis. Granulomatous diseases of unproved etiology. Connective tissue diseases. Elastic tissue diseases. Vasculitis
Scleroderma, Systemic - complications
Scleroderma, Systemic - drug therapy
Scleroderma, Systemic - physiopathology
Small coronary artery disease
Systemic sclerosis
Vasodilator Agents - therapeutic use
Vasodilators
Young Adult
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Summary:The majority of patients with SSc are believed to have subclinical primary cardiac involvement. Overt cardiac manifestations of SSc are associated with poor prognosis and can be difficult to manage. Primary myocardial disease, i.e. without systemic or pulmonary hypertension and without significant pulmonary or renal disease, is postulated to be due to microvascular ischaemia. Undetected early cardiac manifestations can progress silently to myocardial fibrosis. Symptoms may manifest without warning and can rapidly lead to arrhythmia and left and right heart dysfunction and failure. Of the currently practical screening methods, annual echocardiography and/or evaluation of N-terminal portion of pro-B-type natriuretic peptide concentrations should therefore be employed in SSc patients, in order to anticipate the development of cardiac symptoms. Although there is limited evidence in respect of specific therapeutic options, treatment of early abnormalities with calcium channel blockers and angiotensin-converting enzyme inhibitors may improve myocardial perfusion and function, while standard management of overt cardiac disease is equally appropriate in the SSc population. However, it remains to be seen if early intervention can limit the progression of these life-threatening complications.
ISSN:1462-0324
1462-0332
DOI:10.1093/rheumatology/kep110