Sclerodermic Cardiomyopathy—A State-of-the-Art Review

Adrian Giucă,Ruxandra Jurcuț,Ciprian Jurcuţ,Diana Monica Gȋrniţă,Tea Gegenava,Nina Ajmone Marsan,Carmen Marina Mihai,Adrian Săftoiu,Bogdan Alexandru Popescu

doi:10.3390/diagnostics12030669

Abstract

Systemic sclerosis (SSc) is a chronic autoimmune disorder with unknown triggering factors, and complex pathophysiologic links which lead to fibrosis of skin and internal organs, including the heart, lungs, and gut. However, more than 100 years after the first description of cardiac disease in SSc, sclerodermic cardiomyopathy (SScCmp) is an underrecognized, occult disease with important adverse long-term prognosis. Laboratory tests, electrocardiography (ECG) and cardiovascular multimodality imaging techniques (transthoracic 2D and 3D echocardiography, cardiac magnetic resonance (CMR), and novel imaging techniques, including myocardial deformation analysis) provide new insights into the cardiac abnormalities in patients with SSc. This state-of-the-art review aims to stratify all the cardiac investigations needed to diagnose and follow-up the SScCmp, and discusses the epidemiology, risk factors and pathophysiology of this important cause of morbidity of the SSc patient.

Highlights

Systemic sclerosis (SSc) is a rare autoimmune disorder characterized by the following triad: microvascular damage, initial innate and adaptive immune response alterations, and further development of fibrosis in skin and several internal organs [1]
Meune et al compared 129 SSc patients with left ventricle ejection fraction (LVEF) < 55% with 256 SSc patients and LVEF ≥ 55% and found the following independent factors associated with LV dysfunction: male sex (OR: 3.48; 95% C.I. 1.74–6.98), age (OR: 1.03; 95% C.I. 1.01–1.06), digital ulcers (OR: 1.91; 95% C.I. 1.05–3.5), and myositis (OR: 2.88; 95% C.I. 1.15–7.19), while calcium channel blockers (CCB) appeared to have a protective effect (OR: 0.41; 95% C.I. 0.22–0.74) [24]
Presence/severity of arrhythmias does not correlate with skin subtype/symptoms [39]

Summary

Introduction

Systemic sclerosis (SSc) is a rare autoimmune disorder characterized by the following triad: microvascular damage, initial innate and adaptive immune response alterations, and further development of fibrosis in skin and several internal organs [1]. LcSSc patients are characterized by skin thickening limited to the face, neck, and extremities distal to knees and elbows [4]. In dcSSc, skin fibrosis involves at least one proximal region (thorax, abdomen, back, and proximal segments of the limbs) [4]. While in lcSSc, visceral damage is mostly limited and quite stable over time, with Raynaud’s phenomenon (RP) preceding the development of the systemic disorder for years, dcSSc associates in most cases rapid and severe skin and internal organ involvement [4]

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