Cardiac complications of systemic sclerosis

Abstract

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.

Systemic Sclerosis-Associated Pulmonary Arterial Hypertension

“…Steen and colleagues (54) demonstrated that thallium perfusion defect scores were the single most powerful predictors of mortality. Myocardial involvement can also be detected by echocardiography and MRI, and may be present clinically in up to 15 to 35% of patients with SSc (53).…”

Complications and Mortality in Systemic Vasculitis – Vasculogenic Clinicopathologic Entities in Rheumatoid Arthritis and Progressive Systemic Sclerosis Autopsy Patients

“…Studies confirm the increased risk of cardiovascular diseases in RA [4][5][6][7]. The cardiovascular complications-beside renal and pulmonary ones -are important in the mortality of SSc patients as well [8][9][10].…”

Is early diagnosis of pulmonary arterial hypertension possible in inflammatory rheumatic diseases? Experience from a single center in Turkey

“…Treatment options may also vary according to the different types of rheumatic diseases. For instance, immunosuppressive treatment has been shown to be effective in SLE-associated PAH and the response to CCB is poor in rheumatic disease-associated with PAH particularly in SSc (19,27). The response to CCB is poorly defined in other rheumatic diseases.…”