• Masako Tsukamoto, Katsuya Suzuki, Kazuyuki Tsunoda, Kazuhiro Ikeura, Kaori Kameyama, and Tsutomu Takeuchi.
• Division of Rheumatology, Department of Internal Medicine, Keio University School of Medicine, Tokyo, Japan.
• Int J Rheum Dis. 2020 Aug 1; 23 (8): 1024-1029.

Aim/IntroductionAlthough anti-centromere antibody (ACA)+ Sjögren's syndrome (SS) is considered a subtype of SS, it was not included in the recent American College of Rheumatology/ European League Against Rheumatism (ACR/EULAR) SS classification criteria. ACA+ patients without anti-SS-A/Ro antibodies require salivary gland histopathology to fulfill the ACR/EULAR criteria for diagnosis of SS. We reviewed salivary gland histology among ACA+ patients referred for the diagnosis of SS using the ACR/EULAR and Japanese criteria which does not require biopsy.MethodData from 147 ACA+ patients with dry eyes and/or mouth who visited our department were retrospectively analyzed. Clinical, immunological, and histological data were collected and statistically analyzed.ResultSixty-five patients (44%) had undergone labial salivary gland biopsy. The frequency of dry mouth was higher in ACA+ patients who had undergone labial salivary gland biopsy than in those who had not (P = .046), while there were no differences in biopsy rates between patients with and without sclerodactyly (P = .51). According to the current ACR/EULAR classification criteria, Greenspan grade of 3 or 4 for labial salivary gland histopathology is required in patients without anti-SS-A/Ro antibody for the diagnosis of SS. Four patients with Greenspan grades <3 and anti-SS-A/Ro antibody met the criteria for SS. In 54 patients in which the ACR/EULAR criteria were met, 53 patients were diagnosed with SS using the Japanese criteria.ConclusionIn ACA+/anti-SS-A/Ro- antibody patients, agreement between ACR/EULAR and Japanese criteria sets was excellent. For easily classifying ACA+ patients as SS cases, salivary gland biopsy should be performed in ACA+ patients with dry symptoms to identify ACA+ patients.© 2020 Asia Pacific League of Associations for Rheumatology and John Wiley & Sons Australia, Ltd.

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