Increased cancer incidence in SICCA syndrome patients positive for anti-centromere antibody

Abstract

Objectives: The main purposes of this study were to determine the clinical significance of the presence or absence of the anti-centromere antibody in SICCA Syndrome and to diagnose the symptoms. Methods: We retrospectively investigated the prevalence of ACA in patients with SICCA syndrome between January 2006 and January 2014 in Kaohsiung Chang Gung Memorial Hospital. We compared ACA-positive patients with ACA-negative patients. Results: Clinical manifestations in ACA-positive patients are shown in table 2. Many of the clinical symptoms were primarily associated with the predominant disease, scleroderma, limited scleroderma, systemic lupus erythematosus or Sjogren's syndrome (SS). Myalgia was the most prevalent manifestation among those that were ACA-positive. There were fewer ACA-positive SICCA patients with anti-SSA or anti-SSB antibodies than ACA-negative SICCA patients (p ＜0.01, p = 0.011, respectively). Among 35 ACA-positive patients, SS was found in 21 patients (60.0 %), including 13 with primary SS and 8 with secondary SS. In the other 14 patients, 8 had cancer and 6 had other diseases. Among 36 ACA-negative patients, SS was found in 33 patients (91.6 %), including 29 with primary SS and 4 with secondary SS. In the other three patients, one had cancer and two had thyroid diseases. The incidence of cancer was significantly higher in the ACA-positive group than in the ACA-negative group [odds ratio = 10.37; 95 % confidence interval (CI), 1.22-88.02; p ＜ 0.05]. Conclusion: In our study, ACA-positive patients with SICCA syndrome had increased incidence of cancer (p ＜ 0.01; odds ratio = 10.37) and lower prevalence of anti-SSA/anti-SSB antibodies. With regard to cancer risk, anti-centromere positive SICCA syndrome patients should be carefully evaluated for early diagnosis of cancer.