Primary systemic sclerosis heart involvement: A systematic literature review and preliminary data-driven, consensus-based WSF/HFA definition

Abstract

Introduction: Primary heart involvement in systemic sclerosis may cause morpho-functional and electrical cardiac abnormalities and is a common cause of death. The absence of a clear definition of primary heart involvement in systemic sclerosis limits our understanding and ability to focus on clinical research. We aimed to create an expert consensus definition for primary heart involvement in systemic sclerosis. Methods: A systematic literature review of cardiac involvement and manifestations in systemic sclerosis was conducted to inform an international and multi-disciplinary task force. In addition, the nominal group technique was used to derive a definition that was then subject to voting. A total of 16 clinical cases were evaluated to test face validity, feasibility, reliability and criterion validity of the newly created definition. Results: In total, 171 publications met eligibility criteria. Using the nominal group technique, experts added their opinion, provided statements to consider and ranked them to create the consensus definition, which received 100% agreement on face validity. A median 60(5–300) seconds was taken for the feasibility on a single case. Inter-rater agreement was moderate (mKappa (95% CI) = 0.56 (0.46–1.00) for the first round and 0.55 (0.44–1.00) for the second round) and intra-rater agreement was good (mKappa (95% CI) = 0.77 (0.47–1.00)). Criterion validity showed a 78 (73–84)% correctness versus gold standard. Conclusion: A preliminary primary heart involvement in systemic sclerosis consensus-based definition was created and partially validated, for use in future clinical research.

Serum Organ-Specific Anti-Heart and Anti-Intercalated Disk Autoantibodies as New Autoimmune Markers of Cardiac Involvement in Systemic Sclerosis: Frequency, Clinical and Prognostic Correlates

“…The study included consecutive SSc patients with clinically suspected HInv, followed up at the Rheumatology Department, Azienda Ospedaliera Universitaria Careggi (Florence, Italy), and at the Unit of Immunology, Rheumatology, Allergy, and Rare Diseases, IRCCS San Raffaele Hospital (Milan, Italy). Clinically suspected HInv was defined as possessing one or more of the following features: palpitation, dyspnoea or syncope, abnormal troponin I or natriuretic peptides levels, abnormal 12-lead ECG, 24-h ECG Holter monitoring, or echocardiographic findings [4]. All patients underwent CMR.…”

“…It is often clinically silent, and when symptomatic, carries a poor prognosis, accounting for one third of total deaths [2]. Primary HInv is associated with a variable phenotype: clinical presentations include dyspnoea, palpitations, arrhythmias, chest pain, heart failure (HF) with depressed left ventricular ejection fraction (LVEF), and diastolic dysfunction, although most patients are asymptomatic at early stages [3,4]. SSc may have a high arrhythmic burden [5], with a 5% sudden death rate in patients with both skeletal and cardiac muscle diseases [6].…”

Consensus on the assessment of systemic sclerosis–associated primary heart involvement: World Scleroderma Foundation/Heart Failure Association guidance on screening, diagnosis, and follow-up assessment

“…These recommendations refer to the definition of systemic sclerosisrelated primary heart involvement (SSc-pHI) 9 12 8.42 17…”

Cardiotoxicity in autologous haematopoietic stem cell transplantation for systemic sclerosis

“…Primary or secondary SSc-HI 50 has been described in all anatomic sites of the heart (Figure 2), is associated with a significantly increased risk of death. 51,52 Those with rapidly progressive skin thickening or diffuse disease are at highest risk of SSc-HI; these same disease characteristics are indications for AHSCT and hence the AHSCT population may be enriched for SSc-HI.…”