SAT0031 Clinicopathological Characteristics of Anti-Centromere Antibody- And/Or Anti-Ssa Antibody-Positive SjÖGren's Syndrome
Authors: Y. Suzuki, H. Fujii, R. Hamano, I. Mizushima, K. Yamada, M. Kawano
Journal: Annals of the Rheumatic Diseases (2014)

<h3>Background</h3> Several small clinical studies have reported that anti-centromere antibody (ACA)-positive Sjögren9s syndrome (SS) differs from anti-SSA antibody (SSA)-positive SS in some clinical points [1,2]). However, the clinical role of ACA in SS is still unclear. To study the clinical features of ACA-positive SS, 3 groups should be compared; ACA-positive and SSA-positive SS (ACA+SSA+ group), ACA-negative and SSA-positive SS (ACA-SSA+ group), and ACA-positive and SSA-negative SS (ACA+SSA- group). However no reports have ever been designed to compare these 3 groups. There are no reports about ACA+SSA+ SS either. <h3>Objectives</h3> We aimed to examine the clinical role of ACA in SS by clarifying the clinicopathological features of ACA- and/or SSA-positive SS. <h3>Methods</h3> We evaluated 15 patients with ACA+SSA+ SS, 67 patients with ACA-SSA+ SS, and 24 patients with ACA+SSA- SS. All patients met the Japanese Ministry of Health revised criteria (1999) and/or the American College of Rheumatology classification criteria (2012). Patients who had collagen diseases other than systemic sclerosis and had a low titer of SSA were excluded. We performed minor labial salivary gland biopsy in all patients, evaluated focus scores, and compared the clinicopathological data of these 3 groups. Serum levels of many cytokines were also estimated using multiplex assays. In the assays, patients who were being treated with corticosteroid were excluded. <h3>Results</h3> In the 2 ACA-positive groups, age at onset of SS and positive rate for Raynaud9s phenomenon were higher than those of ACA-SSA+ SS. Sclerodactyly was observed in 13.3% of the ACA+SSA+ group, 37.5% of the ACA+SSA- group, and none of the ACA-SSA+ group. No patient developed new skin sclerosis during the follow-up period of mean 40 months. Follow-up period did not significantly differ among the 3 groups. Saxon9s test in the ACA+SSA+ group was lower than that in the ACA-SSA+ group (0.43±0.35 vs 1.39±1.55 g, p=0.009). The 2 SSA-positive groups had lymphocytopenia and high serum IgG levels. The focus scores and fibrosis of the biopsy specimens did not differ significantly. Two ACA+SSA+ patients had pulmonary arterial hypertension. Other extraglandular involvement including malignant lymphoma showed no significant differences in the 3 groups. Serum IL-12 was high in the ACA+SSA+ group, while IFN-γ was high in the 2 SSA-positive groups. <h3>Conclusions</h3> Clinicopathological data and serum cytokine profile in the 3 groups differed. Although the 2 ACA-positive groups had some features of systemic sclerosis such as Raynaud9s phenomenon, not many patients showed sclerodactyly at diagnosis and no patient developed new skin sclerosis during the follow-up. The ACA+SSA+ group may be more likely to have features of SS than those of systemic sclerosis because they showed a lower salivary flow rate in Saxon9s test than that in the ACA-SSA+ SS which was typical SS. Serum IL-12 was high in the ACA+SSA+ group, and there are reports that IL-12 transgenic mice mimic human Sjögren9s syndrome [3]. Thus, measuring ACA is an important component in the management of SS. <h3>References</h3> J Rheumatol 2001;28:2238-44. J Autoimmun 2012;39:15-26. Arthritis Rheum 2009;60:3633-41. <h3>Acknowledgements</h3> We thank John Gelblum for his critical reading of the manuscript. <h3>Disclosure of Interest</h3> None declared <h3>DOI</h3> 10.1136/annrheumdis-2014-eular.3149