Background Anti-centromere antibodies (ACA) are characteristic autoantibodies which are detected in patients with systemic sclerosis (SSc) and primary Sjögren’s syndrome (pSS). We previously reported that ACA are a useful marker of esophageal dilatation in patients with SSc (EULAR2010).
Objectives The aim of this study was to examine the role of ACA for esophageal dysfunction in patients with SSc and pSS using the barium esophagogram during long-term follow up.
Methods Twelve ACA sero-positive pSS (ACA+/pSS) and 20 ACA sero-positive SSc (ACA+/SSc) patients were studied. Barium esophagogram was performed in all ACA+/pSS and ACA+/SSc patients by receiving 4 successive swallows of 20 ml of barium at 30 sec intervals in the standing position. The esophageal dilatation was examined by measurement of maximal diameters at lower esophagus by a single blinded observer. All ACA+/pSS and ACA+/SSc patients were evaluated for the presence of cutaneous subtype, interstitial pneumonia, joint involvement, chronic thyroiditis, primary biliary cirrhosis (PBC) and Raynoud’s phenomenon. We also analyzed γ-globulin, rheumatoid factor (RF), anti-SS-A antibodies, anti-SS-B antibodies, anti-Topoisomerase I antibodies (Topo-I) and anti-U1 RNP antibodies in serum from all patients. The diagnosis of PBC was based on liver function test, the presence of serum anti-M2 antibodies and histopathological findings.
Results All ACA+/pSS and ACA+/SSc patients were women. Follow-up period (6.88±1.98 vs 7.14±1.63 years) and median age (64.8±11.2 vs 59.5±10.6 years) were not different between ACA+/pSS and ACA+/SSc patients. There was no significant difference between ACA+/pSS and ACA+/SSc patients in the prevalence of interstitial pneumonia, joint involvement, chronic thyroiditis, PBC, hyper γ-globulinemia and positivity of RF. However, the prevalence of anti-SS-A antibody and anti-SS-B antibody sero-positive patients were significantly higher in ACA+/pSS than in ACA+/SSc (66.7% vs 20.0%, p=0.008, 25.0 vs 0.0%, p=0.019). Anti-Topo-I antibodies and anti-U1 RNP antibodies were not detected in all patients. Although the esophageal diameters were not significantly different between these groups at the initial evaluation (22.1±2.5mm vs 26.6±8.4mm), they were significantly wider in ACA+/SSc patients than in ACA+/pSS patients at the end of follow-up (21.1±4.1mm vs 30.8±10.5mm, p=0.005). In addition, ACA+/SSc patients showed significant increase of the percentage change of esophageal diameters during follow-up when compared with ACA+/pSS patients (95.3% vs 119.5%, p=0.047).
Conclusions The present study demonstrated that esophageal dilatation was progressive in ACA sero-positive patients during long-term follow-up. However, the severity of esophageal dilatation depended on the underlying diseases. Another factor other than ACA seems to contribute to esophageal dysfunction.
Disclosure of Interest None Declared