Value of labial salivary gland histopathology for diagnosis of Sjögren's syndrome in patients with anti‐centromere antibody positivity

Abstract

Although 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. Data 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. Sixty-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. In 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.