Background/Purpose: Cardiac involvement in systemic sclerosis (SSc) is one of the leading causes of scleroderma-associated death. There are limited data examining risk factors for incident cardiomyopathy or for cardiac recovery in patients with SSc.

Methods: Patients in a large North American longitudinal scleroderma cohort with at least two echocardiograms were included. We identified predictor variables associated with incident cardiomyopathy (ejection fraction (EF) ≥ 50% declining to < 50%) and severe cardiomyopathy (EF > 35% dropping to ≤ 35%) by fitting two multivariate logistic regression models. Time-varying and invariant variables were used to model EF transitioning from one state to another. For time-varying variables, values captured most recently prior to each EF transition were used. Variables that associate with cardiac recovery (EF < 50% to ≥ 50%, EF ≤ 35% to > 35%) were identified by fitting multivariate logistic regression models using important variables identified from random forest models. We reviewed the medical records of the 139 patients whose EF ≤ 35% was ever to further characterize the etiology of severe cardiomyopathy.

Results: 2,303 patients in the cohort with at least two echocardiograms were included, and 13,209 echocardiograms were analyzed. Male sex, Black race, diffuse skin involvement, onset age, higher mRSS, RVSP ≥ 45, kidney disease, and atrial fibrillation were associated with increased odds of incident cardiomyopathy (EF < 50%), while anti-centromere and anti-Scl70 were protective (Table 1). Male sex, anti-Ku, higher mRSS, RVSP ≥ 45, skeletal myopathy, kidney disease, and atrial fibrillation were associated with higher odds of incident severe cardiomyopathy (EF ≤ 35%). When previous EF was below 50%, tendon friction rubs were associated with lower odds of cardiac recovery, and anti-RNA polymerase III (POLR3) with higher odds of cardiac recovery (Table 2). When previous EF was less than or equal to 35%, diabetes was associated with lower odds of recovering to EF > 35%, and anti-POLR3 with higher odds. The most common causes of EF ≤ 35% identified through medical record review were ischemic heart disease, conduction/arrhythmia-related, myocarditis, fibrosis, right heart failure from pulmonary hypertension, infection, and renal crisis. Representative trajectories of patients with scleroderma-related cardiomyopathy are shown in Figure 1.

Conclusion: This study provides the most detailed analysis of factors associated with varying degrees of incident cardiomyopathy in SSc using time-varying predictor variables and is the only study to explore factors associated with left ventricular recovery. Distinct demographic, scleroderma-specific and cardiac risk factors associate with increased risk of incident cardiomyopathy in scleroderma. Anti-POLR3 antibodies were associated with EF recovery.

« Back to ACR Convergence 2024

ACR Meeting Abstracts - https://acrabstracts.org/abstract/identification-of-risk-factors-for-incident-cardiomyopathy-in-patients-with-systemic-sclerosis/