Background/Purpose: Anti-centromere antibody (ACA) positive Sjögren’s syndrome (SS) is considered as a subtype in SS. Recent international collaborative large scale cohort study highlighted several clinical features such as Raynaud’s phenomenon、 sclerodactyly and extra glandular dysfunction (Arthritis Care Res. 2016). Assessment of ACA is potentially valuable for definitive diagnosis of this subtype and medical management in a certain number of patients uncovered by current 2015 ACR/EULAR classification criteria. However, enough information of clinical and immunological features of ACA positive SS has not been accumulated and clinical significance of ACA in SS may not be fully established. The aim of this study is to clarify clinical and immunological features of ACA positive SS.

Methods: All patients with primary SS (pSS) who visited to our Division of Rheumatology at Keio University Hospital in Tokyo between May 1995 and December 2016 were enrolled. Clinical information and immunological tests including immunoglobulin (Ig) and serum autoantibodies were collected and statistically analyzed.

Results: Five hundred and ninety nine patients were clinically classified as pSS (female: 93%, mean age: 55 ± 15). They were divided into 4 groups by serum ACA and anti-SS-A antibody status. Only discrete-speckled pattern in anti-nuclear antibody (ANA) test and/or anti-centromere antibody positive (ACA alone) were detected in 39 patients (6.5%), while only anti-SS-A antibody with no ACA (SS-A alone) were detected in 449 patients (75.0%). Number of patients with both ACA and SS-A (Double positive) was 40 (6.7%), while 67 patients had neither ANA nor SS-A (Seronegative). Then we statistically compared these 4 groups. The proportions of Raynaud’s phenomenon or sclerodactyly were higher in ACA alone and Double positive groups (p<0.01 or p<0.01). The proportion of dryness was no difference among 4 groups. The proportions of increase of serum IgG or IgA were 15% or 7% in ACA alone group, 61% or 20% in SS-A alone group, 50% or 28% in Double positive group and 22% or 4% in Seronegative group (p<0.01 or p<0.01). Existence of anti-SS-A antibody, not ACA associated to high concentration of IgG or IgA, while there was no difference between 4 groups as IgM (p=0.40). Regarding C3, C4 or CH50, there were no differences among 4 groups. Remarkably, the proportion of leukocytopenia in ACA alone group was significantly higher than the others (p=0.02). As compared with pulmonary, cardiac or articular involvements, no differences were found among 4 groups. Importantly the proportion of patients who had facial erythema was significantly lower in ACA positive SS (p<0.01).

Conclusion: Our large-scale study identified distinct characteristics of ACA-positive SS patients different from anti-SS-A/Ro antibody positive or seronegative SS patients in Japanese population.

ACR Meeting Abstracts - https://acrabstracts.org/abstract/distinct-clinical-and-immunological-features-of-anti-centromere-antibody-positive-primary-sjogrens-syndrome-a-single-center-cross-sectional-cohort-study/