Presentation: Cardiac Involvement in Systemic Sclerosis: The Strongest Predictive Factor of Prognosis in Patients with Scleroderma (2007)

13 Cardiac Involvement in Systemic Sclerosis: The Strongest Predictive Factor of Prognosis in Patients with Scleroderma

Purpose: We analyzed 159 patients with scleroderma to clarify the frequency and characteristics of cardiac involvement in the early stage of disease, and explored how cardiac involvement affects the survival.
Patients and Methods: A hundred fifty-nine consecutive Japanese patients with scleroderma (diffuse: 134, limited: 25) underwent evaluation of our hospital from January 1995 to September 2006. Anti Scl-70 and anti centromere antibodies were detected in 57 and 27 patients, respectively. Electrocardiogram and chest X-ray were performed in all patients, and ultrasonic echocardiography in 92 patients as an initial evaluation. Based on these examinations, we diagnosed heart involvement. For survival analysis, we performed a retrospective cohort study. The primary outcome was death. Survival was measured from the date of the initial evaluation.
Results: The median age at the initial evaluation, which was performed within 5 years from disease onset was 57.9 (IQR: 16.2, 79.6) years. Cardiac involvement was detected in 29 patients. Out of these 29 patients, arrhythmia, myocardial involvement and pericarditis were found in 21 patients (72.4%), 14(48.3%), 16 (55.1%), respectively. Bradycardiac arrhythmia (including in atrioventricular block) were diagnosed in 9 patients, and tachycardic arrhythmia (including in premature ventricular contraction (Lown’s class ≥3), premature supraventricular contraction and atrial fibrillation) in 14 patients. It is notable that no patient had symptom except for a patient with sick sinus syndrome. Fifty-seven patients died during the observation period for long as 11.7 years. The survival ratio was significantly lower in patients with cardiac involvement rather than in patients without cardiac involvement (mean±SEM years: 4.2±0.9 vs. 8.2±0.4, respectively, P< 0.001, Fig.). As shown in table, cardiac involvement indicated the highest and significant hazard ratio for death among various organ involvement at the initial evaluation.
Conclusions: The results disclosed that most frequent cardiac manifestation at the initial evaluation of scleroderma is subclinical arrhythmia. More importantly, cardiac involvement at early stage of the disease is the strongest predictive factor for death.
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Table. multivariate analysis using Cox proportional hazards model
Table. multivariate analysis using Cox proportional hazards model
covariateHR for death95%CIp
female (n=144)0.6780.264 - 1.7390.419
age (y)1.0291.004 - 1.0540.021
diffuse type (n=134)1.5350.639 - 3.6860.338
interstitial lung disease (n=96)1.4980.784 - 2.8620.221
pulmonary hypertension (n=30)2.0861.176 - 3.7000.012
cardiac involvement (n=29)3.6172.012 - 6.502<0.001
scleroderma renal crisis (n=5)2.2710.646 - 7.9840.201
gastrointestinal involvement (n=36)1.2470.639 - 3.6860.338

 S. Tanaka, None; K. Nishi, None; N. Iizuka, None; H. Kondo, None; S. Hirohata, None.