Background Primary Sjögren syndrome (pSS) shares many clinical, inflammatory, and immunological features with rheumatoid arthritis (RA) and systemic lupus erythematosus (SLE). Both SLE and RA are characterized by a high risk of cardiac involvement. However, there are limited data on the risk of overt cardiac involvement in pSS.
Objectives We sought to use a cardiac magnetic resonance imaging (CMR) approach to assess cardiac involvement and determine its association with disease characteristics in pSS patients without cardiac symptoms.
Methods Consecutive pSS patients, according to ACR classification criteria (2012), without a history or clinical findings of hypertension, cardiovascular disease, diabetes, or dyslipidemia underwent contrast CMR. Late gadolinium enhancement (LGE) was used for the assessment of myocardial fibrosis. Using a black-blood T2-weighted image (T2-WI), myocardial inflammation could be assessed. Sjögren syndrome disease activity index (ESSDAI) was determined. Eighty percent patients had documentation of a minor salivary gland biopsy. Salivary gland biopsy data were classified by focus score (FS). We investigated the patients in terms of prevalence of CMR abnormalities and explored possible associations between CMR abnormalities and pSS disease characteristics.
Results Thirty-seven female pSS patients were enrolled (mean age: 55.5 ± 7.0 years). On an average, cardiovascular risk was low for the group, with patients demonstrating no ECG abnormalities, and the patients had generally low traditional cardiovascular risk factors, with a mean Framingham 10-year hard cardiovascular risk score of 4 ± 2%. The mean ESSDAI was 3.3 ± 2.1. Thirteen patients (35%) demonstrated myocardial abnormalities. Myocardial edema was seen in 5 patients (13%) on T2-WI. LGE was found in 11 patients (29%), 3 of whom demonstrated edema on T2-WI. The main finding observed in 7 among 11 LGE-positive patients (63%) was a linear LGE pattern without coronary distribution. A patchy nodular LGE pattern was observed in 4 among 11 patients (37%). The patients with CMR abnormalities showed no significant difference of ESSDAI, compared with those with no CMR abnormalities. Antibodies to La/SSB antigens were significantly higher in LGE-positive than LGE-negative patients (p=0.003). Raynaud phenomenon was significantly associated with LGE-positive and T2-WI-positive patients (p=0.001 and p=0.04, respectively). Other pSS characteristics such as disease duration, commodities, and cardiovascular risk factors were not significantly associated with myocardial abnormalities. The greatest relative difference between LGE-positive and -negative patients was observed in FS >3, with an adjusted odds ratio of 3.0. After adjusting for confounding by age, pSS duration, and anti-SSB antigen, the association of LGE with Reynaud phenomenon remained significant (p=0.02).
Conclusions Subclinical myocardial involvement, as detected by CMR, was frequent in pSS patients without cardiac symptoms. Our results suggest that Raynaud phenomenon has a role in promoting cardiac involvements in patients with pSS.
Disclosure of Interest None declared