Abstract
BackgroundTo clarify the clinicopathological characteristics of primary Sjögren's syndrome (pSS) with anti-centromere antibody (ACA).MethodsCharacteristics of 14 patients of pSS with ACA were evaluated. All patients were anti-SS-A/Ro and SS-B/La antibodies negative (ACA+ group) without sclerodactyly. The prevalence of Raynaud's phenomenon (RP), titer of IgG and focus score (FS) in the minor salivary glands (MSGs) were determined. Quantification analysis of Azan Mallory staining was performed to detect collagenous fiber. Forty eight patients in whom ACA was absent were chosen as the conventional (ACA-) pSS group.ResultsPrevalence of ACA+ SS patients was 14 out of 129 (10.85%) pSS patients. RP was observed in 61.5% of the patients with ACA. The level of IgG in the ACA+ group was significantly lower than that of the ACA- group (p = 0.018). Statistical difference was also found in the FS of MSGs from the ACA+ group (1.4 ± 1.0) as compared with the ACA- group (2.3 ± 1.6) (p = 0.035). In contrast, the amount of fibrous tissue was much higher in the ACA+ group (65052.2 ± 14520.6 μm2 versus 26251.3 ± 14249.8 μm2 ) (p = 1.3 × 10-12).ConclusionsLow cellular infiltration but with an increase in fibrous tissues may explain the clinical feature of a high prevalence of RP and normal IgG concentration in ACA+ pSS.
Highlights
To clarify the clinicopathological characteristics of primary Sjögren's syndrome with anticentromere antibody (ACA)
Our present data confirmed the previous observation that ACA+ primary Sjögren's syndrome (pSS) has a high prevalence of Raynaud' s phenomenon (RP) despite normal IgG, which is different from ACApSS, which is seropositive toward anti-s syndrome (SS)-A/Ro Ab or anti-SS-B/La Ab [5,8]
Since there was no statistical difference with regard to followup period between ACA+ and ACA- pSS groups in this study, we suggest that the high prevalence and normal IgG in ACA+ pSS is specific for this disease subset
Summary
To clarify the clinicopathological characteristics of primary Sjögren's syndrome (pSS) with anticentromere antibody (ACA). Primary Sjögren's syndrome (pSS) is characterized by sicca symptoms and various extraglandular manifestations that are usually accompanied by autoantibodies, especially anti-SS-A/Ro and SS-B/La antibodies (Abs) [1]. Recent studies [5,6] have demonstrated that ACA is associated with pSS. The prevalence of anti-SS-A/Ro or SS-B/La Abs in pSS is 60-70%, ACA is reported to appear in 1626% of patients with pSS. Some reports [7] have shown the clinical characteristics of ACA-positive (ACA+) pSS, and a high frequency of Raynaud' s phenomenon (RP) has been repeatedly reported. In addition to the low prevalence of RP, Katano et al [8] reported a low titer of IgG in an ACA+ and anti-SS-A/Ro antibody-negative pSS population
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