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Objective: To assess the serum level of immunoglobulin A rheumatoid factor (IgA-RF) in patients with primary Sjogren'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.

Primary Sjögren's syndrome (pSS) is a connective tissue disease with symptoms and serological findings often overlapping with systemic lupus erythematosus (SLE) (1). Thromboembolic events are common in SLE but not in pSS (2)(3). However, case reports have described pSS patients who developed fulminant multiorgan disease due to thrombotic diathesis 4, and we have presented a case with acute catastrophic anti-phospholipid syndrome (APS) in a pSS patient (5). In this study we wanted to examine the incidence of thromboembolic episodes and relate these to the presence of autoantibodies and coagulation abnormalities in 90 pSS patients during a 4.6-year follow-up.

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.

Background: The impact of primary Sjögren's syndrome (pSS) on reproduction and gynaecological manifestations has seldom been explored., Aim of Study: Assess gynaecological aspects, gynaecological interventions, and use of contraceptives in a population of pSS-patients versus controls., Methods: In a case-control study, 58 pSS-patients and 157 controls answered a self-administered questionnaire, covering demographic data, reproductive events, gynaecological problems, and gynaecological interventions., Results: Significantly more patients than controls reported episodes of amenorrhoea lasting for more than 3 months, and more patients suffered from menorrhagia/metrorrhagia compared with controls (54.5% versus 35.7%; p = 0.012). Complaints of vaginal dryness were common among the patients (52.9% versus 28.3%; p = 0.005). Endometriosis was reported to occur more frequently in the patients (8.5% versus 2.1%; p = 0.03), and 6.3% of pSS-patients reported having undergone surgical intervention for endometriosis versus 0.7% of the controls (p = 0.009). Positive information about surgery for endometriosis correlated with the presence of the autoantibodies anti-SSA (r = 0.322; p = 0.008) and anti-SSB (r = 0.313; p = 0.01). Among the pSS-patients, 5.9% had chosen not to have children due to the disease, but there was no indication of reduced fertility as judged by the number of pregnancies., Conclusion: Patients with pSS reported more gynaecological problems than controls, including vaginal sicca symptoms, endometriosis, several episodes of amenorrhoea, and menorrhagia/metrorrhagia.

Objective: To determine if calprotectin is predictive for outcome in patients with rheumatoid arthritis (RA)., Methods: Fifty six RA in-patients with variable disease duration were prospectively followed for five years. Clinical and laboratory data were collected to assess disease activity. Health Assessment Questionnaire (HAQ) and radiographic scores (of hands and wrists) as described by Larsen were used as outcome measures. Plasma calprotectin levels were determined with ELISA technique., Results: Significant correlations (r) were found cross-sectionally at follow-up between calprotectin concentration and other known parameters of disease activity and severity: CRP (r = 0.67). investigator's global assessment of disease activity (r = 0.57). Waaler titre (r = 0.50). HAQ score (r = 0.48) and number of swollen joints (r = 0.48). Calprotectin at baseline was not identified as an independent predictor for HAQ or radiographic progression in the multivariate analysis., Conclusion: The results confirm calprotectin as a good measure of disease activity and joint inflammation in RA. However, the level of calprotectin at baseline was not predictive for radiographic damage or functional impairment five years later.

Objective: To investigate and compare the accuracy and usefulness of diagnostic tests for rheumatoid factor (RF)., Methods: In a cross-sectional study sera derived from patients admitted to the Section of Rheumatology were tested for presence of RF using either nephelometry or the Waaler test. Diagnostic sensitivity and predictive values of the tests were calculated and compared. The accuracy of the tests was compared using receiver-operating characteristics (ROC) methodology., Results: Good agreement was found between the tests (kappa approximately 0.7). At cut-off 19 IU/mL nephelometry showed the highest sensitivity (82.4%) and specificity (95.9%) for rheumatoid arthritis (RA). In comparison, the Waaler test had a sensitivity of 60.3% and specificity of 95.9% at cut-off titer 128. The tests showed nearly equal performance characteristics when predicting SS., Conclusion: Although both tests exhibit good performance characteristics, nephelometry has a higher accuracy when predicting RA and SS. The common practice of using both tests for detection of RF is not recommended.

A patient with primary Sjögren's syndrome developed pulmonary embolism following infection with influenza A virus. IgM anti-cardiolipin autoantibodies (aCL) evolved two weeks after hospitalisation, synchronously with antibodies against influenza A. IgG aCL developed three weeks after hospitalization, peaked during the recovery period, and gradually declined to undetectable levels 12 months after admission. Antibodies against beta2 glycoprotein I were not detected. Our results assign a high likelihood to the hypothesis that influenza A virus caused the patient's thromboembolic disease as well as development of aCL. aCL may have contributed to tissue pathology by forming immune-complexes with cardiolipin and rheumatoid factor.

Intracranial lipoma is a rare condition, and it is usually asymptomatic. We describe a 67 year old woman who developed blurred vision, diplopia, left sided oculomotor palsy, and ipsilateral ptosis during steroid treatment for giant cell arteritis. These symptoms were considered to be associated with aggressive giant cell arteritis, and the steroid dose was raised. Surprisingly, the symptoms increased, and further examination revealed an intracranial lipoma situated in the Meckel's cave. During tapering of the steroids her symptoms gradually improved. This is the first report demonstrating that steroids may induce hypertrophy of the fat tissue in the intracranial lipoma, causing compression of the cranial nerves passing through the cavernous sinus thereby mimicking the ocular symptoms sometimes associated with aggressive giant cell arteritis.

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.

The prevalence of thyroid antibodies in 100 patients with rheumatoid arthritis in Northern Norway was studied. The serological data were compared with those from a major population survey in the same area. Compared to the prevalence in the normal population, the present study demonstrates that patients with rheumatoid arthritis have a higher prevalence of antibodies to both thyroid microsomal antigen and thyroglobulin.