American-European Consensus Group Classification Criteria Research Articles

Meibomian gland dysfunction and primary Sjögren’s syndrome dry eye: a protocol for systematic review and meta-analysis

IntroductionPrimary Sjögren’s syndrome (pSS) is a systemic autoimmune disorder that primarily affects the exocrine glands such as the lacrimal and the salivary glands. Dry eye disease (DED) is one of...

Ultrasonographic characteristics of major salivary glands in anti-centromere antibody-positive primary Sjögren's syndrome.

To investigate salivary gland ultrasonography (SGUS) findings in primary Sjögren's syndrome (pSS) patients positive for the anti-centromere antibody (ACA) and compare these with those in ACA-negative pSS patients. We analyzed demographic, clinical, laboratory, and SGUS data of pSS patients who fulfilled the 2002 American-European Consensus Group classification criteria for pSS. SGUS findings of four major salivary glands (bilateral parotid and submandibular glands) were scored in five categories and compared between ACA-positive and ACA-negative pSS patients. Linear regression analysis was performed to elucidate the factors associated with SGUS score. In total, 121 pSS patients were enrolled (19, ACA-positive). The ACA-positive patients were older (67.0 vs 58.0 years, P = 0.028), whereas anti-Ro/SSA and anti-La/SSB positivity was more prevalent in the ACA-negative group (89.2% vs 21.1%, P < 0.001, and 47.1% vs 10.5%, P = 0.007, respectively). The total SGUS and hypoechoic area scores were lower in ACA-positive patients (16.0 vs 23.0, P = 0.027, and 4.0 vs 7.0, P = 0.004, respectively). In univariate regression analysis, being positive for unstimulated salivary flow rate (USFR < 1.5 ml/15 min), anti-Ro/SSA, and rheumatoid factor were positively associated whereas ACA positivity was negatively associated with the SGUS score. In multivariate regression analysis, being positive for USFR, anti-Ro/SSA, and rheumatoid factor showed significant association with the SGUS score. ACA-positive pSS patients showed a lower SGUS score than ACA-negative patients, which was especially prominent in the hypoechoic area component.

Ten-year survival analysis of patients with primary Sjögren's syndrome in China: a national prospective cohort study.

Aims:To investigate the long-term survival of patients with primary Sjögren’s syndrome (pSS) in China.Methods:Patients with pSS who fulfilled the 2002 American–European Consensus Group classification criteria were prospectively enrolled from 2004 to 2011. Their baseline clinical, laboratory, and therapeutic data were collected. The primary endpoint was all-cause death by January 2018. The standard mortality ratio (SMR) was calculated by comparing with age-matched and sex-matched mortality data of the general population. Kaplan–Meier curves were obtained by time-to-event analysis. Univariate and multivariate Cox hazards regression analyses were performed to identify risk factors for mortality.Results:A total of 1054 patients were enrolled and 834 patients were followed up for a median of 94.8 months, with 48 confirmed deaths. The total SMR was 3.63 [95% confidence interval (CI) 2.60–4.66]. The 3-, 5-, and 10-year survival rates were 98.4%, 97.5%, and 92.9%, respectively. Infection, malignancy, and respiratory failure were the top three causes of mortality. We identified male sex [hazard ratio (HR) = 3.00, 95% CI 1.23–7.31], age at diagnosis ⩾50 years of age (HR = 7.69, 95% CI 3.01–19.62), thrombocytopenia (HR = 1.93, 95% CI 1.01–3.72), and interstitial lung disease (HR = 5.96, 95% CI 2.24–15.82) as the independent prognostic factors of death.Conclusions:The mortality rates of Chinese patients with pSS are higher than those of the general population. Male patients of elder age at diagnosis complicated with thrombocytopenia and interstitial lung disease might be suggestive for poorer survival and require closer follow up.

The Prevalence of Sjögren's Syndrome in Rheumatoid Arthritis Patients and Their Clinical Features.

BackgroundTo estimate the prevalence of Sjögren's syndrome (SS) in patients with rheumatoid arthritis (RA) and to compare the clinical features of RA patients with and without SS.MethodsWe conducted a retrospective study of RA patients who visited a rheumatology clinic in a tertiary referral hospital in Korea between May 20 and July 22, 2016. All patients fulfilled the classification criteria for RA, and the diagnosis of SS was made clinically by rheumatologists and according to the 2002 American-European Consensus Group (AECG), 2012 American College of Rheumatology (ACR), and 2016 ACR/European League Against Rheumatism (EULAR) classification criteria. The prevalence was estimated as the number of SS patients within the total number of RA patients. The disease activity and treatment pattern of RA were compared between patients with and without SS.ResultsAmong 827 RA patients, 72 patients (8.7%) were diagnosed with SS by a rheumatologist, though only 60 patients (7.3%) satisfied the 2002 AECG classification criteria for SS. Fifty-two patients (6.3%) and 56 patients (6.8%) fulfilled the 2012 ACR and 2016 ACR/EULAR classification criteria, respectively. The prevalence of SS in RA patients was 10.5%, 17.0%, and 67.6% in rheumatoid factor, antinuclear antibody (≥ 1:80), and anti-Ro antibody positive patients, respectively.ConclusionThe prevalence of SS among RA patients was 8.7% according to rheumatologists' diagnosis. The presence of SS did not affect the treatment patterns of RA patients. However, the autoantibody profiles and demographics of RA patients with SS differed from those of patients without SS.

Unique Sjögren's syndrome patient subsets defined by molecular features.

To address heterogeneity complicating primary SS (pSS) clinical trials, research and care by characterizing and clustering patients by their molecular phenotypes. pSS patients met American-European Consensus Group classification criteria and had at least one systemic manifestation and stimulated salivary flow of ⩾0.1 ml/min. Correlated transcriptional modules were derived from gene expression microarray data from blood (n = 47 with appropriate samples). Patients were clustered based on this molecular information using an unbiased random forest modelling approach. In addition, multiplex, bead-based assays and ELISAs were used to assess 30 serum cytokines, chemokines and soluble receptors. Eleven autoantibodies, including anti-Ro/SSA and anti-La/SSB, were measured by Bio-Rad Bioplex 2200. Transcriptional modules distinguished three clusters of pSS patients. Cluster 1 showed no significant elevation of IFN or inflammation modules. Cluster 2 showed strong IFN and inflammation modular network signatures, as well as high plasma protein levels of IP-10/CXCL10, MIG/CXCL9, BLyS (BAFF) and LIGHT. Cluster 3 samples exhibited moderately elevated IFN modules, but with suppressed inflammatory modules, increased IP-10/CXCL10 and B cell-attracting chemokine 1/CXCL13 and trends toward increased MIG/CXCL9, IL-1α, and IL-21. Anti-Ro/SSA and anti-La/SSB were present in all three clusters. Molecular profiles encompassing IFN, inflammation and other signatures can be used to separate patients with pSS into distinct clusters. In the future, such profiles may inform patient selection for clinical trials and guide treatment decisions.

Whole-Volume ADC Histogram Analysis in Parotid Glands to Identify Patients with Sj\xf6gren\u2019s Syndrome

At present, no gold standard for diagnosing Sjögren’s syndrome (SS) is available in clinical practice. The 2002 American–European Consensus Group classification criteria are used to diagnose SS. Clinically, it is challenging to distinguish patients with SS from suspected patients undergoing different therapies. A total of 52 patients with SS and 24 patients suspected of having the disease prospectively underwent 3.0-T magnetic resonance (MR) scanning, including diffusion-weighted imaging (b = 0 and 1000 s/mm2). The whole-volume apparent diffusion coefficient (ADC) histogram analysis generated ADCmean, skewness, kurtosis, and entropy values from bilateral parotid glands. Continuous variables were compared using an independent two-sample t test, and categorical variable compared using the Fisher’s test between the two groups. Receiver operating characteristic (ROC) analysis was used to evaluate the diagnostic performance of the indexes. Fisher’s tests demonstrated that some clinical indexes and MR morphology grades differed significantly between patients with SS and patients suspected of having the disease (all P ≤ 0.001). The parotid entropy value of patients with SS was significantly higher than that of patients suspected of having the disease (P < 0.001). Among MR parameters, entropy combined with kurtosis performed the best in differentiating patients with SS from those suspected of having SS (area under the ROC curve = 0.955). A whole-volume ADC histogram analysis might provide a series of parameters that reflect tissue characteristics.

Activation of Toll-like receptor 7 signaling in labial salivary glands of primary Sjögren's syndrome patients.

The aim of this study was to determine the expressions of Toll-like receptors (TLRs) 7-9 and type I interferon (IFN) signal in labial salivary glands (LSGs) and cultured salivary gland epithelial cells (SGECs) from primary Sjögren's syndrome (pSS) patients. We performed an immunohistochemistry analysis of LSGs from 11 patients with pSS as defined by American-European Consensus Group classification criteria and five healthy subjects. The pSS patients' SGECs were analyzed by immunofluorescence and western blotting. IFN-α expression was examined by immunosorbent assay and flow cytometry. Mononuclear cells (MNCs) from pSS patients' LSGs showed TLR-7-dominant expression. B cells, plasma cells and plasmacytoid dendritic cells (pDCs) co-expressed with TLR-7. Myeloid differentiation primary response gene 88 (MyD88), tumor necrosis factor receptor-associated factor 6 (TRAF6) and interferon regulatory factor 7 (IRF7) co-expressed with the pDC marker CD303 in LSGs. Ducts from pSS patients dominantly expressed TLR-7, and TLR-7 in the ducts co-expressed with MyD88, TRAF6 and IRF7. Type I IFNs including IFN-α and IFN-β were detected in MNCs and ducts in pSS patients' LSGs. Increased TRAF6 expression and the nuclear translocation of IRF7 in SGECs were detected by immunofluorescence following loxoribine (a TLR-7 ligand) stimulation despite IFN-β pretreatment. Western blotting showed increased TRAF6 expression in SGECs following IFN-β and loxoribine stimulation. Although no increase in IFN-α was detected in supernatant from stimulated SGECs, the IFN-α in supernatant from stimulated peripheral blood pDCs from pSS patients was significantly increased. Our findings suggest that TLR-7 is dominantly expressed in both MNCs and ducts with downstream signals for type I IFNs, indicating that TLR7-dominant innate immunity is related to the development of sialadenitis in pSS.

Ultrasound elastography in assessment of salivary glands involvement in primary Sjögren's syndrome

ObjectiveOur study aimed to assess the pathological sonoelastographic changes in the major salivary glands and to demonstrate the diagnostic effectiveness of Sonoelastography as an additional method to US in Sjögren's syndrome. MethodsFifty eight patients with primary Sjögren's syndrome (pSS) were selected according to the American-European Consensus Group Classification criteria. Twenty five healthy volunteers involved in this study. All patients were evaluated with B-mode and elastography by using Hitachi EUB 7500 digital ultrasound equipment. All subjects were female. The sonoelastography examination, which allowed us to assess the elasticity of the parenchyma, was performed and strain ratios were measured by comparing with the adjacent tissues. ResultsStatistically significant differences were found between the pSS and control groups for the elastographic scores and strain ratios (p < 0.001). The highest sensitivity and specificity levels were obtained when the strain ratio cut-off value was taken as 1.55 for the submandibular gland and 2.45 for the parotid gland (sensitivity and specificity were 83% and 88% respectively for the submandibular gland and 83% and 92% respectively for the parotid gland). However, no statistically significant differences were found between the disease duration and the elasticity scores or strain ratios in pSS group (p > 0.05). ConclusionUS examination is an efficient method to assess major salivary gland involvement in the diagnosis of pSS. Sonoelastography is a modality which can contribute to the diagnosis by improving specificity in the differential diagnosis of pSS. Strain ratio measurement, which is a semi-quantitative method, increases the diagnostic effectiveness by providing high sensitivity, specificity, and negative predictive values.

Characteristics and risk factors of pulmonary arterial hypertension in patients with primary Sjögren's syndrome.

To describe baseline characteristics of patients with primary Sjögren's syndrome (pSS) with right heart catheterization (RHC)-confirmed pulmonary arterial hypertension (PAH) and explore risk factors for PAH in pSS. This case-control study included consecutive patients hospitalized with pSS-PAH from 2007 to 2015, and pSS patients without PAH (in a 4 : 1 ratio) as controls. All patients fulfilled the 2002 American-European Consensus Group classification criteria for pSS-PAH was defined according to RHC-based European Society of Cardiology/European Respiratory Society guidelines. Associated variables were analyzed by univariate binary logistic regression to identify possible risk factors for PAH. Twenty-nine patients with RHC-confirmed pSS-PAH were included (mean age at onset, 38.4 ± 8.3 years; mean pSS duration, 54.6 months). PAH was the initial manifestation of pSS in 12 patients (41.4%), and shortness of breath was the most common symptom (29/29, 100%). Mean pulmonary arterial pressure was 51.8 ± 10.0 mmHg, mean cardiac index was 2.3 ± 0.8 L/min/m2 , and mean pulmonary vascular resistance was 13.0 ± 6.0 Wood units in this group. Treatments included immunosuppressive agents (93.1%) and PAH-targeted therapies (86.2%). We identified four independent risk factors for PAH in pSS: Raynaud's phenomenon (odds ratio [OR] = 9.660, P = 0.000), rheumatoid factor ≥ 200 U/mL (OR = 6.691, P = 0.001), hepatic injury (OR = 3.284, P = 0.008) and pericardial effusion (OR = 3.279, P = 0.016). PAH can be the first manifestation of pSS. The pSS patients with Raynaud's phenomenon, high-titer rheumatoid factor, hepatic injury or pericardial effusion should be screened for PAH.

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

ObjectiveTo 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...

Comparison of performance of the 2016 ACR-EULAR classification criteria for primary Sjögren's syndrome with other sets of criteria in Japanese patients

ObjectivesTo compare the performance of the new 2016 American College of Rheumatology (ACR)-European League Against Rheumatism (EULAR) classification criteria for primary Sjögren's syndrome (SS) with 1999 revised Japanese Ministry of...

Usefulness of IgA Anti-α-fodrin Antibodies in Combination with Rheumatoid Factor and/or Antinuclear Antibodies as Substitute Immunological Criterion in Sjögren Syndrome with Negative Anti-SSA/SSB Antibodies.

We aimed to evaluate the usefulness of anti-α-fodrin antibodies (AFA) in combination with rheumatoid factor (RF) and/or antinuclear antibodies (ANA) as an alternative immunological criterion for Sjögren syndrome (SS) among patients with negative anti-Ro/La serology. The study included 350 patients (100 with rheumatoid arthritis, systemic lupus erythematosus, and systemic sclerosis, and 50 with primary SS) randomly selected and assessed for SS. All patients were tested for ANA, RF, anti-SSA/SSB, and AFA antibodies. SS diagnosis was made on a clinical basis by 2 rheumatologists based on the 6-item screening questionnaire, Schirmer-I test, nonstimulated whole salivary flow rate, fluorescein staining test, autoantibodies, lip biopsy, and medical chart review. Non-SS was defined as lack of clinical diagnosis and not fulfilling the American-European Consensus Group classification criteria and the American College of Rheumatology (ACR) criteria. The ACR criteria were applied substituting the immunological criteria as follows: (1) RF plus ANA > 1:320, (2) RF plus AFA, (3) ANA > 1:320 plus AFA, (4) RF alone, and (5) 2 positive tests out of RF, ANA > 1:320, or AFA. We estimated the sensitivity, specificity, positive predictive value, negative predictive value, and likelihood ratio positivity with 95% CI for each criterion. There were 236 patients (67%) who tested negative for anti-SSA/SSB antibodies, of whom 65 (27.5%) were clinically diagnosed as SS, and 149 (63%) with non-SS. RF + AFA and ANA + AFA performed similarly to RF + ANA > 1:320. The model 2 out of 3 of RF, ANA, or AFA improved the sensitivity from 56.9% to 70.7%, although the specificity decreased. The combination AFA + RF, AFA + ANA > 1:320, or at least 2 out of 3, performed well as a proxy immunological test for patients with SS and negative Ro/La serology.

Reevaluation for clinical manifestations of HTLV-I-seropositive patients with Sjögren's syndrome.

BackgroundThe aim of the study was to reassess the prevalence and characteristics of human T lymphotropic virus type I (HTLV-I)-associated Sjögren’s syndrome (SS) and SS in HTLV-I-associated myelopathy (HAM) based on the American European Consensus Group (AECG) criteria in HTLV-I endemic area, Nagasaki prefecture.MethodsThe 349 patients who underwent a minor salivary gland biopsy (MSGB) for suspected SS were retrospectively classified by AECG classification criteria and divided with or without anti-HTLV-I antibody.ResultsThe HTLV-I data-available 294 patients were investigated. One hundred-seventy patients were classified as SS and 26.5 % were HTLV-I-seropositive. We have included 26 patients with HTLV-I-associated myelopathy (HAM) and 38.5 % were classified as having SS. The prevalences of ANA and anti-SS-A/Ro antibody of HAM + SS were significantly low compared to the HTLV-I asymptomatic carriers (AC) with SS and the HTLV-I-seronegative SS patients, although lacrimal dysfunction tended to be high in HAM + SS and significantly high in AC + SS patients compared with the patients with HTLV-I-seronegative SS. The focus scores of MSGB in the HAM + SS patients were similar to those of the AC + SS patients and the HTLV-I-seronegative patients with SS. Among the MSGB-positive patients, there was a low prevalence of ANA in the HAM + SS patients. Similar results were obtained in case of anti-SS-A/Ro or SS-B/La antibody.ConclusionIn HTLV-I endemic area, high prevalence of anti-HTLV-I antibody among SS as well as the characteristics of HAM + SS and AC + SS was still determined by AECG classification criteria.

FRI0435 Salivary Epidermal Growth Factor (EGF) in SjÖGren's Syndrome: Association between Salivary EGF Levels and the Severity of Intraoral Manifestations

BackgroundXerostomia accompanied in patients with Sjögren's syndrome (SS) relates to reduced salivary flow rate as a result of salivary gland dysfunction. Dry mouth causes various intraoral manifestations such as refractory...

FRI0373 Clinical Outcome of Dental Implant Therapy in Patients with SjÖGren's Syndrome

BackgroundPatients with Sjögren's syndrome are subject to loss of teeth related to the high caries risk. Dental implant therapy is often considered as a proper possibility for prosthodontic rehabilitation of...

Primary and secondary surveys on epidemiology of Sjögren's syndrome in Japan

Objective. To characterize the epidemiology of Sjögren's syndrome (SS), including prevalence, disease type, extra-glandular involvement, satisfaction of diagnostic criteria sets, and treatment used in Japan.Methods. The Research Team for Autoimmune Diseases, the Research Program for Intractable Disease by the Ministry of Health, Labor and Welfare conducted primary and secondary surveys on epidemiology of SS in 2011. The primary survey covered 4,729 out of 14,095 Japan-wide Hospital Departments to investigate the prevalence of SS. The secondary survey encompassed 214 Hospital Departments that agreed to the survey, to characterize disease type, extra-glandular involvement, satisfaction of diagnostic criteria sets, and treatments.Results. The number of patients with SS in Japan estimated by the primary survey was 68,483. The secondary survey involving data collected from 2,195 SS patients from 98 Hospital Departments showed that the mean age of patients was 60.8 ± 15.2 years, male/female ratio was 1/17.4, primary/secondary SS was about 60%/40% and glandular/extra-glandular form in primary SS was about 70%/25%. The satisfaction rate was 53.8% for the 1999 revised Japanese Ministry of Health criteria for the diagnosis of SS, 47.7% for the 2002 American–European Consensus Group classification criteria for SS and 49.6% for 2012 American College of Rheumatology classification criteria for SS. Corticosteroids were used by 752 of 2,195 patients (34%), immunosuppressants by 358 patients (16%), biologics by 68 patients (3%) and secretagogues by 695 patients (32%).Conclusion. The surveys provided valuable information on the epidemiology of SS including prevalence, disease type, extra-glandular involvement, satisfaction of diagnostic criteria sets and treatments used today in Japan.

SP0113 Primary SjöGren’s Syndrome: What is New in 2012 to 2013? (Win)

The last year, significant progress has been made in the diagnostics, evaluation and treatment of primary Sjögren’s syndrome (pSS). The progress in the various areas will be discussed in detail.With...

CXC ligand 13 in rheumatoid arthritis and its relation to secondary Sjögren’s syndrome

Aim of the workThe aim of the present study was to measure the level of the chemokine CXC ligand 13 protein (CXCL13) in the plasma and unstimulated saliva of rheumatoid arthritis (RA) patients in order to find out its role in the disease activity and its relation to secondary Sjögren’s syndrome (sSS). Patients and methodsThe study was conducted on thirty rheumatoid arthritis patients attending the Outpatient Clinic of Rheumatology and Rehabilitation department of Ain shams University Hospitals. The patients’ group had been classified into group (1) which included fifteen RA patients associated with sSS diagnosed according to the American–European Consensus Group Classification Criteria and group (2) which included fifteen RA patients not associated with sSS. Ten healthy subjects were included as a control group. Patients were subjected to full history taking, clinical examination, and laboratory detection of CXCL13 level in the plasma and saliva of patients as well as the control groups using ELISA technique. Assessment of disease activity in RA patients was done using the disease activity score (DAS28). ResultsPlasma levels of CXCL13 were significantly higher in RA patients than control group (p<0.001). Plasma levels of CXCL13 were significantly correlated with the RA disease activity (r=0.677, p<0.001) and disease duration (r=0.406, p<0.05), while the salivary levels were higher in those with sSS and correlated with sSS disease duration (r=0.536, p<0.05). A highly significant correlation was found between salivary CXCL13 and severity of sSS (r=0.816, p<0.001). Salivary levels of CXCL13 above 110pg/ml may diagnose sSS with sensitivity 80% and specificity 84%. ConclusionThe results of this preliminary study point out the importance of CXCL13 as a marker for RA disease activity, its role in diagnosing sSS, and estimation of sSS severity.

Validation of different sets of criteria for the diagnosis of Sjögren’s syndrome in Japanese patients

Objective: To validate the revised Japanese Ministry of Health criteria for the diagnosis of Sjögren’s syndrome (SS) (JPN) (1999), The American-European Consensus Group classification criteria for SS (AECG) (2002), and American College of Rheumatology classification criteria for SS (ACR) (2012).Methods: The study subjects were 694 patients with SS or suspected SS who were followed-up in June 2012 at ten hospitals that form part of the Research Team for Autoimmune Diseases, The Research Program for Intractable Disease by the Ministry of Health, Labor and Welfare (MHLW). All patients had been checked for all four criteria of the JPN (pathology, oral, ocular, anti-SS-A/SS-B antibodies). We studied the clinical diagnosis made by the physician in charge and the satisfaction of the above criteria.Results: Of the 694 patients, 499 patients did not have other connective tissue diseases (CTDs). SS was diagnosed in 476 patients (primary SS in 302, secondary SS in 174), whereas non-SS was diagnosed in 218 patients (without other CTDs in 197, with other CTDs in 21) by the physician in charge. The sensitivities of JPN, AECG, and ACR in the diagnosis of all forms of SS (both primary and secondary SS) were 79.6, 78.6, and 77.5 %, respectively, with respective specificities of 90.4, 90.4, and 83.5 %. The sensitivities of the same systems in the diagnosis of primary SS were 82.1, 83.1, and 79.1 %, respectively, with specificities of 90.9, 90.9, and 84.8 %, respectively. The sensitivities of the same systems in the diagnosis of secondary SS were 75.3, 70.7, and 74.7 %, respectively, with specificities of 85.7, 85.7, and 71.4 %, respectively.Conclusion: The sensitivity of JPN to all forms of SS and secondary SS, the sensitivity of AECG to primary SS, and the specificities of JPN and AECG for all forms of SS, primary SS, and secondary SS were highest in the diagnosis of SS in Japanese patients. These results indicate that the JPN criteria for the diagnosis of SS in Japanese patients are superior to ACR and AECG.

Validation of different sets of criteria for the diagnosis of Sjögren’s syndrome in Japanese patients

To validate the revised Japanese Ministry of Health criteria for the diagnosis of Sjögren's syndrome (SS) (JPN) (1999), The American-European Consensus Group classification criteria for SS (AECG) (2002), and American College of Rheumatology classification criteria for SS (ACR) (2012). The study subjects were 694 patients with SS or suspected SS who were followed-up in June 2012 at ten hospitals that form part of the Research Team for Autoimmune Diseases, The Research Program for Intractable Disease by the Ministry of Health, Labor and Welfare (MHLW). All patients had been checked for all four criteria of the JPN (pathology, oral, ocular, anti-SS-A/SS-B antibodies). We studied the clinical diagnosis made by the physician in charge and the satisfaction of the above criteria. Of the 694 patients, 499 patients did not have other connective tissue diseases (CTDs). SS was diagnosed in 476 patients (primary SS in 302, secondary SS in 174), whereas non-SS was diagnosed in 218 patients (without other CTDs in 197, with other CTDs in 21) by the physician in charge. The sensitivities of JPN, AECG, and ACR in the diagnosis of all forms of SS (both primary and secondary SS) were 79.6, 78.6, and 77.5%, respectively, with respective specificities of 90.4, 90.4, and 83.5%. The sensitivities of the same systems in the diagnosis of primary SS were 82.1, 83.1, and 79.1%, respectively, with specificities of 90.9, 90.9, and 84.8%, respectively. The sensitivities of the same systems in the diagnosis of secondary SS were 75.3, 70.7, and 74.7%, respectively, with specificities of 85.7, 85.7, and 71.4%, respectively. The sensitivity of JPN to all forms of SS and secondary SS, the sensitivity of AECG to primary SS, and the specificities of JPN and AECG for all forms of SS, primary SS, and secondary SS were highest in the diagnosis of SS in Japanese patients. These results indicate that the JPN criteria for the diagnosis of SS in Japanese patients are superior to ACR and AECG.