Impact of double positive for anti-centromere and anti-SS-a/Ro antibodies on clinicopathological characteristics of primary Sj€ogren's syndrome: a retrospective cohort study.

Objectives: The purpose of our study was to define the clinical characteristics of anti-centromere antibody and anti-SS-A/Ro antibody (ACA/SS-A) double positive Sj€ogren's syndrome (SS) and to clarify the clinical impact of these antibodies. Methods: We examined 108 patients (6 males, mean age 57.9 years) with SS who underwent labial salivary gland biopsy. The patients were divided into four groups by ACA and anti-SS-A/Ro antibody positivity. Symptoms, laboratory and pathological data, and scleroderma-related data were compared among the groups. Results: The cohort consisted of 16 ACA/SS-A double positive, 20 ACA single positive, 67 SS-A single positive, and 5 ACA/SS-A double negative SS. ACA/SS-A double positive SS were significantly older than SS-A single positive SS (mean age 71.1 vs. 53.1 years). They had higher EULAR Sj€ogren's syndrome disease activity index (ESSDAI) at diagnosis (mean 3.81 vs. 0.50) and higher serum IgG (mean 2009 vs. 1389mg/dL) than ACA single positive SS. No patients developed skin sclerosis during a mean followup period of 45.6 months (range: 1-178). Conclusion: These results demonstrate that ACA/SS-A double positive SS is distinct from ACA single positive and SSA single positive SS. The combination of ACA and anti-SS-A/Ro antibody in SS should deserve greater attention in clinical practice. [ABSTRACT FROM AUTHOR]