Preliminary European Criteria Research Articles

The evolution of international classification criteria for Sjogren’s syndrome

This article presents review of literature of the history of international classification criteria of American College of Rheumatology/European Alliance of Associations for Rheumatology (ACR/EULAR) 2016, considers the features of validation and development of preliminary European criteria 1993, criteria of American European Consensus Group (AECG) 2002 and ACR 2012 criteria.

Classification criteria of Sjögren's syndrome

Sjögren's syndrome (SS) is a chronic, systemic autoimmune disease that affects typically the exocrine glands causing mucosal dryness. Dry eyes and mouth are considered by far the most common and early symptoms of the disease but systemic complications may also occur. In 1993, the preliminary European criteria were proposed and widely accepted, consisting of both subjective and objective criteria. Almost ten years later, these classification criteria were revised by introducing more stringent rules and precise diagnostic procedures leading to the currently used American-European Consensus Group (AECG) criteria. The AECG criteria have been largely employed to conduct epidemiologic and clinical studies of patients with SS and proved to be more specific compared to the preliminary European criteria. The recent American College of Rheumatology/Sjögren's International Collaborative Clinical Alliance (ACR/SICCA) criteria that are based exclusively on objective tests, the stringency of the AECG criteria and the potential therapeutic use of biologic agents in SS clearly set the need for new classification criteria. Whether the new diagnostic approach will further encompass subclinical and early forms of the disease remains to be addressed by the scientific community.

IgA rheumatoid factor in primary Sjögren's syndrome

Objective: To assess the serum level of immunoglobulin A rheumatoid factor (IgA‐RF) in patients with primary Sjögren's syndrome (pSS) and study the association with immunological and clinical factors.Methods: Sera from 97 pSS patients diagnosed according to the preliminary European criteria and 100 controls were analysed for IgA‐RF in a cross‐sectional study design.Results: IgA‐RF was detected in serum of 25.8% of the pSS patients and in 1% of the controls. In patients with positive vs. negative IgA‐RF, the focus scores in biopsy of the minor salivary glands were 4.41 and 1.43 (p<0.0001), respectively. There was a correlation between positive IgA‐RF and positive antinuclear antibodies (ANA) (r = 0.263, p<0.009), IgM‐RF (r = 0.70, p<0.0001), anti‐SSA/SSB (r = 0.73, p<0.0001), and a high serum level of IgG (r = 0.59, p<0.0001). The presence of renal disease was higher in IgA‐RF‐positive vs. negative pSS patients (20.0% vs. 5.6%, p = 0.047). The serum level of the hormone prolactin (PRL) correlated to the serum level of IgA‐RF (r = 0.31, p = 0.026).Conclusions: The presence of IgA‐RF in patients with pSS is closely associated with the presence of autoantibodies, and with focus scoring in biopsies of the salivary glands. IgA‐RF is associated with renal disease in pSS but we found no correlation to other extraglandular manifestations.

Diagnostic evaluation and classification criteria in Sjögren's Syndrome

Objectives Our objective is to carry out a clinical study of the performance of the preliminary European classification criteria for Sjögren Syndrome and that of the criteria proposed by the American European Consensus Group. Methods Eighty-eight patients who had undergone a biopsy of the salivary gland on suspicion that they were suffering from Sjögren Syndrome were studied by two independent rheumatologists. Two pathologists independently revised the biopsies without knowing the diagnosis. With all of this information, the clinicians established, or did not establish, a diagnosis of primary Sjögren Syndrome or secondary Sjögren Syndrome. Finally, it was analysed whether or not the patients met the American European Consensus Group classification criteria and the preliminary European criteria for primary Sjögren Syndrome and secondary Sjögren Syndrome, and their sensitivity and specificity with respect to the clinical diagnosis were determined. Results Clinicians estimated that 35 cases (39.8%) had primary Sjögren Syndrome (kappa 0.718) and 17 cases (19.3%) had secondary Sjögren Syndrome (kappa 0.761). The specificity and sensitivity of American European Consensus Group criteria, with regard to the clinical diagnosis, for primary Sjögren Syndrome were 97.2% and 48.6%, respectively. For secondary Sjögren Syndrome, the specificity was 97.2% and the sensitivity 64.7%. The preliminary European criteria for primary Sjögren Syndrome demonstrated a lesser specificity (75%), but a higher sensitivity (65.7%). In secondary Sjögren Syndrome the specificity reached 97.2% with sensitivity at 70.6%. Conclusions These results underline the difficulty in applying the Sjögren Syndrome classification criteria from the American European Consensus Group and the preliminary European criteria, in the diagnosis of individual patients.

Estimation of the prevalence of primary Sjögren's syndrome in two age‐different community‐based populations using two sets of classification criteria: the Hordaland Health Study

Objective: To estimate the point prevalence of primary Sjögren's syndrome (pSS) in two populations, aged 40–44 and 71–74 years, using two sets of classification criteria.Methods: The participating individuals were recruited from the Hordaland Health Study (HUSK) conducted during 1997–99. A total of 18 592 individuals born 1953–57 and 3346 individuals born 1925–27 were sent a questionnaire covering various health‐related questions, including four questions about sicca symptoms. Among those answering positive to at least one of the four questions, 99 and 90 individuals born 1953–57 and 1925–27, respectively, were examined further. For diagnosis of pSS two classifications were used, the preliminary European criteria from 1993, and the revised European criteria from 1996.Results: By using the two classification criteria from 1993 and 1996, the point prevalences were 0.44% [95% confidence interval (CI) 0.34–0.57] and 0.22% (95% CI 0.15–0.32), respectively, for the population group born 1953–57. The corresponding estimates were 3.39% (95% CI 2.77–4.14) and 1.40% (95% CI 1.02–1.92) for the population born 1925–27.Conclusion: The point prevalence of pSS was approximately seven times higher in the elderly population aged 71–74 years compared to individuals aged 40–44 years, regardless of the classification criteria used.

LE SYNDROME DE GOUJEROT-SJÖGREN PRIMITIF

Objective : To assess clinical and biological correlation of systemic manifestations of primary Sjogren’s syndrome (SS). The diagnosis of our patients used the preliminary european criteria established in 1993. Material and methods: It’s a retrospective study about thirty tree patients with primary SS are documented (32 women and 1 men; men age: 45, 3 years). Results : The systemic manifestations are present in 91% and they were reasons for hospital referral in 42% of cases. They included 85% of particular signs, 21% of muscular manifestations and 24% of Raynaud phenomenon. Pulmonary involvements found in 45% cases, neurological manifestations in 15% and renal disorder in 30% cases. Antinuclear antibodies were found in 52% and rheumatoid factor in 60%. Conclusion : Systemic manifestations are frequent in our series. There incidence are similar with the literature, except for the neurological involvement with is rare.

A prospective study of sicca symptoms in patients with rheumatoid arthritis.

To investigate sicca symptoms in patients with rheumatoid arthritis (RA) with respect to constancy, temporal changes of prevalence, and possible risk factors. A prospective cohort study of 70 patients with RA was conducted over 5 years. The main variables of interest were the 6 questions on sicca symptoms used in the preliminary European criteria for Sjögren's syndrome. Fourteen patients were lost to followup. We found that 84.2% (95% confidence interval [95% CI] 59.5-95.8) of the patients reporting sicca symptoms at baseline also reported them at followup. During the study period, sicca symptoms increased by 52.6% in general (P = 0.02) and by 80.0% for the ocular components (P = 0.04). Sicca symptoms (odds ratio [OR] = 8.35, 95% CI 1.91-36.49) and pain (OR = 1.03, 95% CI 1.00-1.07) at baseline were identified as independent predictive factors for sicca symptoms at followup. Sicca symptoms in patients with RA are remarkably constant over time. There is also a substantial time-dependent increase in the prevalence of such symptoms. As the prevalence of ocular sicca symptoms in general populations tend to level out with age, there seems to be a disease-related increase of ocular symptoms over time in patients with RA. Present pain and sicca symptoms constitute risk factors for future sicca symptoms.

Sjögren's syndrome in an out-patient clinic: classification of patients according to the preliminary European criteria and the proposed modified European criteria.

To investigate whether out-patients with a clinical diagnosis of Sjögren's syndrome (SS) satisfied current preliminary European criteria for SS, and to determine the proportion of patients who satisfied the proposed modified European criteria for SS and thus had indication of an autoimmune process. Out-patients with a clinical diagnosis of SS registered between 1 January 1999 and 1 November 2000 were included in the study. Of 203 patients with a clinical diagnosis of SS, 116 (57.1%) satisfied the current European criteria and 83 (40.9%) satisfied the proposed modified criteria. Sicca symptoms and signs may have a variety of causes. In our study only 40.9% of the patients with a clinical diagnosis of SS satisfied the proposed modified European criteria and had evidence of SS as an autoimmune condition. Our findings indicate that for patient populations with an established diagnosis of SS according to the preliminary European criteria, approximately one-third will lose the diagnosis according to the modified criteria.

A comparison of salivary gland hypofunction in primary and secondary Sjögren's syndrome

A commonly held view by clinicians is that the salivary gland hypofunction associated with primary Sjögren's syndrome (SS-1) is more severe than that associated with secondary Sjögren's syndrome (SS-2). This study aimed to determine if this view could be substantiated, when applied to a large sample group. Unstimulated and paraffin wax-stimulated whole salivary flow rates were retrospectively compared for age and gender matched, patients diagnosed with SS-1 or SS-2 according to the preliminary European criteria. The patients had attended the Xerostomia Clinic, in the Oral Medicine Department, at the Liverpool University Dental Hospital. Sixty-seven patients with SS-1 (average age 57.1 years) were matched with 67 patients with SS-2 (average age 57.6 years), according to gender and age, within 5 years. The mean unstimulated whole salivary flow rates (+/- s.d.) for patients with SS-1 and SS-2 were 0.11 (+/- 0.15) and 0.12 (+/- 0.18) mL min-1 respectively. The mean paraffin wax stimulated, whole salivary flow rates (+/- s.d.) for patients with SS-1 and SS-22 were 0.45 (+/- 0.02) and 0.47 (+/- 0.49) mL/min-1 respectively. No significant differences, in either stimulated (P = 0.54) or unstimulated (P = 0.60) whole salivary flow rates were found between individuals with SS-1 or SS-2. The severity of salivary gland hypofunction does not appear to be related to the clinical variant of Sjögren's syndrome.

Comparison of the Copenhagen criteria and the preliminary European criteria for primary‐SS

Comparison of the Copenhagen criteria and the preliminary European criteria for primary‐SS

The Influence of Age on Disease Manifestations and Serological Characteristics in Primary Sjögren's Syndrome

We have studied the influences of age on clinical manifestations and serological abnormalities in primary Sjögren's syndrome (pSS). The 67 patients included were diagnosed according to the preliminary European classification criteria for pSS, and disease manifestations were assessed according to a newly developed quantitative and qualitative classification model. We found that the age at diagnosis correlated to the presence of autoantibodies anti-SSA/SSB (r=-0.31, <0.02), rheumatoid factor (RF) (r=-0.33, p<0.01) and serum levels of IgG (r=-0.27, p<0.05). Young patients diagnosed before 45 years of age had the highest positivity of the autoantibodies anti-SSA/SSB (62.5%), RF (62.5%) and high levels of s-IgG (68.8%), while the corresponding values for old patients diagnosed after 60 years of age were anti-SSA/SSB: 20.8%, RF: 20.8%, and high s-IgG: 29.2%. None of these parameters correlated to age at onset nor disease duration. By applying a model for evaluating disease manifestations, no correlation between the global index nor the indices for subgroups of disease manifestations was found to age at onset, age at diagnosis, nor disease duration. We conclude that there is a significant influence of age on serological abnormalities in pSS, but not on disease manifestations.