The cardiac magnetic resonance in the diagnosis of cardiac Raynaud phenomenon in a patient with systemic sclerosis: case report and review of literature

ABSTRACT

Raynaud phenomenon (RP) is the hallmark of Systemic Sclerosis (SSc). Visceral RP has also been proposed in SSc patients. Cardiac Raynaud’s phenomenon (C-RP) was evaluated in a few clinical studies both as cold-induced transient myocardial ischaemia and as presence of advanced myocardial fibrosis and contraction band necrosis in autopsied patients. Until today numerous techniques, such as scintigraphy and myocardial contrast echocardiography, have been used to evaluate C-RP. In this case report for the first time we have used Cardiac Magnetic Resonance (CMR) after cold test to demonstrate the presence of the C-RP. In addition we have shown that therapy with Iloprost can be used to reduce episodes of C-RP.

KEYWORDS:

• Systemic sclerosis
• Cardiac Raynaud’s phenomenon
• Cardiac Magnetic Resonance

Financial and competing interests disclosure

The authors have no relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript. This includes employment, consultancies, honoraria, stock ownership or options, expert testimony, grants or patents received or pending, or royalties.

Key issues

• SSc is a connective tissue disease.

• Microvascular damage of skin and internal organs is a hallmark of SSc.

• Visceral RP has also been proposed in SSc patients.

• C-RP is cold-induced transient myocardial ischemia.

• Chest pain in SSc patients could be triggered by episodes of C-RP.

• The C-RP diagnosis is very difficult.

• The CMR with CT is an useful technique to evaluate C-RP in SSc patients.

• The intravenous infusion of iloprost reduces the frequency and intensity of C-RP episodes.