Background/Purpose:

Congenital heart block in the fetus and neonate, which can cause acquired QT prolongation, may be associated with maternal anti-SS-A/anti-SS-B autoantibodies. However, there are increasing reports that primary Sjögren syndrome (pSS) is associated with an increased risk of cardiovascular disease and that disease-related clinical and immunological markers may promote cardiovascular disease. We hypothesized that myocardial abnormalities were associated with the corrected QT (QTc) interval in pSS. We used cardiac magnetic resonance imaging (CMR) to assess cardiac involvement and determine its association with the QTc interval in pSS patients without cardiac symptoms.

Methods:

Consecutive pSS patients, classified according to the 2012 ACR criteria with no history or clinical findings of hypertension, cardiovascular disease, diabetes, or dyslipidemia underwent contrast-enhanced CMR. Late gadolinium enhancement (LGE) was used to assess myocardial fibrosis. Myocardial inflammation was assessed using a black-blood T2-weighted image (T2-WI). The Sjögren syndrome disease activity index (ESSDAI) was determined. Eighty-six percent patients had documentation of a minor salivary gland biopsy. Salivary gland biopsy data were classified by focus score (FS). A QTc interval of 440 ms was considered as prolonged.

Results:

Fifty-five pSS patients were enrolled (mean age: 53.2±9.6 years). The mean ESSDAI was 2.5±2.7. Myocardial edema was seen in 3 patients (5%) on T2-WI. LGE was found in 9 (16%), 2 of whom demonstrated edema on T2-WI. Raynaud’s phenomenon was significantly associated with LGE-positive patients (p=0.0064). The greatest relative difference between LGE-positive and -negative patients was observed in FS ≥4, with an adjusted odds ratio of 4.0, although the FS was not associated with the QTc interval.

PSS patients had a longer mean QTc interval than did controls (432.5±24.9 vs. 420.5±14.4; p=0.003). Furthermore, there was significant difference in the QTc interval between the LGE-positive and LGE-negative group (447.7±15.7 vs 429.5±25.4; p=0.012). Other pSS characteristics, such as disease duration, anti-SS-A/anti-SS-B autoantibodies, ESSDAI, and cardiovascular risk factors, were not significantly associated with myocardial abnormalities and QTc interval.

A receiver operating characteristic analysis showed that the QTc interval reliably detected myocardial abnormalities (area under the curve, 0.77).

Conclusion:

Subclinical myocardial involvement, as detected by CMR, was frequent in pSS patients without cardiac symptoms. Abnormal CMR findings were associated with a QTc interval. To our knowledge, this was the first study to show that myocardial abnormalities in pSS were associated with the QTc interval.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/the-corrected-qtqtc-interval-is-associated-with-myocardial-fibrosis-in-primary-sjogren-syndrome-assessed-by-a-cardiac-magnetic-resonance-approach-a-prospective-pilot-study-at-a-single-center/