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SAT0031 Clinicopathological Characteristics of Anti-Centromere Antibody- And/Or Anti-Ssa Antibody-Positive SjÖGren's Syndrome
  1. Y. Suzuki,
  2. H. Fujii,
  3. R. Hamano,
  4. I. Mizushima,
  5. K. Yamada,
  6. M. Kawano
  1. Division of Rheumatology, Department of Internal Medicine, Kanazawa University Graduate School of Medicine, Kanazawa, Japan


Background Several small clinical studies have reported that anti-centromere antibody (ACA)-positive Sjögren's 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.

Objectives We aimed to examine the clinical role of ACA in SS by clarifying the clinicopathological features of ACA- and/or SSA-positive SS.

Methods 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.

Results In the 2 ACA-positive groups, age at onset of SS and positive rate for Raynaud's 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. Saxon's 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.

Conclusions 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 Raynaud's 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 Saxon's 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 Sjögren's syndrome [3]. Thus, measuring ACA is an important component in the management of SS.


  1. J Rheumatol 2001;28:2238-44.

  2. J Autoimmun 2012;39:15-26.

  3. Arthritis Rheum 2009;60:3633-41.

Acknowledgements We thank John Gelblum for his critical reading of the manuscript.

Disclosure of Interest None declared

DOI 10.1136/annrheumdis-2014-eular.3149

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