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

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.

Tsukamoto M ，Suzuki K ，Tsunoda K ，Ikeura K ，Kameyama K ，Takeuchi T ... -  《-》

Ultrasonography of major salivary glands compared with parotid and labial gland biopsy and classification criteria in patients with clinically suspected primary Sjögren's syndrome.

To assess the validity of ultrasound of major salivary glands (sUS) compared with parotid and labial gland biopsies, sialometry, anti-SSA/Ro antibody status and classification criteria in patients clinically suspected with primary Sjögren's syndrome (pSS). 103 consecutive outpatients with clinically suspected pSS underwent sUS. Parenchymal echogenicity, homogeneity, hypoechogenic areas, hyperechogenic reflections and clearness of salivary gland border were scored according to the Hocevar scoring system. Total ultrasound score was calculated as the sum of these domains (range 0-48). Absolute agreement between sUS and parotid (83%) and labial (79%) gland biopsy outcome was good. Negative sUS predicts negative parotid gland biopsy, and positive sUS predicts positive labial gland biopsy. Compared with the American European Consensus Group (AECG) classification, sUS showed an absolute agreement of 82%, sensitivity of 71% and specificity of 92%. Compared with the American College of Rheumatology (ACR) classification, absolute agreement was 86%, sensitivity was 77% and specificity was 92%. Compared with the ACR-European League Against Rheumatism (EULAR) classification, absolute agreement was 80%, sensitivity was 67% and specificity was 94%. Positive sUS predicts classification, but negative sUS does not exclude classification. The combination of positive sUS with presence of anti-SSA/Ro antibodies or negative sUS with absence of anti-SSA/Ro antibodies showed a high predictive value for classification as pSS or non-pSS. In our prospective inception cohort study derived from daily clinical practice, absolute agreement between sUS and salivary gland biopsies was slightly higher for parotid compared with labial gland biopsies. The combination of positive sUS and presence of anti-SSA/Ro antibodies highly predicts classification according to the AECG, ACR and ACR-EULAR classification criteria.

Mossel E ，Delli K ，van Nimwegen JF ，Stel AJ ，Kroese FGM ，Spijkervet FKL ，Vissink A ，Arends S ，Bootsma H ，EULAR US-pSS Study Group ... -  《-》

Anti-centromere antibody-positive Sjögren's syndrome: A distinct clinical subgroup?

To investigate whether patients with Sjögren's syndrome (SS) can be distinguished based on the positivity of anti-centromere antibody (ACA), and if so, whether the subgroups differ in their clinical and laboratory features. Eleven patients with ACA-positive and 71 patients ACA-negative SS were examined. All patients had minor salivary gland biopsy; sociodemographic data, glandular and extraglandular manifestations, and laboratory findings, including autoantibodies, complement and immunoglobulin levels, were analyzed. European League Against Rheumatism SS disease activity index (ESSDAI) and SS disease damage index (SSDDI) were also measured. The prevalence of ACA among SS patients was 13.4%. ACA-positive SS patients had a higher prevalence of Raynaud's phenomenon, sclerodactyly and autoimmune thyroiditis and a lower prevalence of anti-SSA/Ro and anti-SSB/La antibodies compared to ACA-negative patients. Disease activity was higher in ACA-positive patients than in ACA-negative patients, but the damage index did not differ between the two groups. None of the patients who originally had ACA evolved to having full-blown systemic sclerosis. Patients with SS who have ACA differ from classic SS patients in several clinical and laboratory parameters. ACA should be considered one of the pathogenically relevant autoantibodies for SS.

Lee KE ，Kang JH ，Lee JW ，Wen L ，Park DJ ，Kim TJ ，Park YW ，Lee SS ... -  《-》

Impact of double positive for anti-centromere and anti-SS-a/Ro antibodies on clinicopathological characteristics of primary Sjögren's syndrome: a retrospective cohort study.

Suzuki Y ，Fujii H ，Nomura H ，Mizushima I ，Yamada K ，Yamagishi M ，Kawano M ... -  《-》

Clinical characteristics and biopsy accuracy in suspected cases of Sjögren's syndrome referred to labial salivary gland biopsy.

Giovelli RA ，Santos MC ，Serrano ÉV ，Valim V ... -  《BMC MUSCULOSKELETAL DISORDERS》