American College of Rheumatology classification criteria for Sjögren's syndrome: a data-driven, expert consensus approach in the Sjögren's International Collaborative Clinical Alliance cohort.

OBJECTIVE: We propose new classification criteria for Sjögren's syndrome (SS), which are needed considering the emergence of biologic agents as potential treatments and their associated comorbidity. These criteria target individuals with signs/symptoms suggestive of SS. METHODS: Criteria are based on expert opinion elicited using the nominal group technique and analyses of data from the Sjögren's International Collaborative Clinical Alliance. Preliminary criteria validation included comparisons with classifications based on the American­European Consensus Group (AECG) criteria, a model-based "gold standard"obtained from latent class analysis (LCA) of data from a range of diagnostic tests, and a comparison with cases and controls collected from sources external to the population used for criteria development. RESULTS: Validation results indicate high levels of sensitivity and specificity for the criteria. Case definition requires at least 2 of the following 3: 1) positive serum anti-SSA and/or anti-SSB or (positive rheumatoid factor and antinuclear antibody titer >1:320), 2) ocular staining score >3, or 3) presence of focal lymphocytic sialadenitis with a focus score >1 focus/4 mm2 in labial salivary gland biopsy samples. Observed agreement with the AECG criteria is high when these are applied using all objective tests. However, AECG classification based on allowable substitutions of symptoms for objective tests results in poor agreement with the proposed and LCA-derived classifications. CONCLUSION: These classification criteria developed from registry data collected using standardized measures are based on objective tests. Validation indicates improved classification performance relative to existing alternatives, making them more suitable for application in situations where misclassification may present health risks.

Winchester syndrome.

Winchester syndrome was first described in 1969 and since then nine patients have been reported in the literature. The syndrome is characterized by short stature, coarse face, corneal opacities, generalized osteolysis and progressive painful arthropathy with joint stiffness and contractures of distal phalanges in combination with skin changes. The etiology is unknown. Parental consanguinity supports autosomal inheritance. The diagnosis is based on clinical and radiological manifestations. We describe a case in a 7-year-old Pakistani boy.

[Still disease in adults]. / Mb. Still hos voksne.

Adult onset Stills disease (ASD), an adult variant of systemic onset juvenile rheumatoid arthritis, is a rare disease entity. The diagnosis is solely a clinical one and often difficult. Clinical and laboratory features are not pathognomonic. The diagnosis of ASD has to be considered in patients with high spiking fever, transient rash, arthralgias, oligo- or polyarticular arthritis, leukocytosis, sore throat, lymphadenopathy and/or splenomegaly, liver dysfunction and high serum ferritin levels. We give a brief review of the clinical features, differential diagnosis, treatment and prognosis.

[Two cases of Still disease in adults]. / To tilfaelde af Morbus Still hos voksne.

We describe two cases of adult onset Stills disease. Both patients presented with typical features of adult Stills disease: high spiking fever, arthralgia, oligo- and polyarticular arthritis, transient rash, sore throat, lymphadenopathy and leukocytosis. Both patients failed to improve when treated with nonsteroidal antiinflammatory drugs (NSAIDs) and azathioprine, but responded adequately when sulfasalazine was added to the medication. It is suggested that sulfasalazine is a useful adjunct if the clinical response to NSAIDs is not sufficient.

[Recurrent arthritis in a child. A rare manifestation of Wilson's disease]. / Recidiverende arthritis hos et barn. En sjoelden manifestation af Wilsons sygdom.

Wilsons disease is a rare autosomal recessive disorder of copper transportation, which is fatal if not treated. The disease often starts in adolescence, and most common symptoms are due to liver-and/or brain involvement. This paper deals with an adolescent with Wilsons disease. His clinical presentation was joint complaints for almost two years. The final diagnosis was made by mutation analysis. It is stressed that the clinician should consider Wilson's disease in cases of unexplained liver- and neurological involvement as well as cases of repetitive unexplained joint symptoms in the pubertal period.

Retroperitoneal fibrosis treated with methylprednisolon pulse and disease-modifying antirheumatic drugs.

The conventional treatment of patients with ureteric obstruction due to retroperitoneal fibrosis (RF) is surgery in combination with long-term corticosteroids. This report describes 11 cases of RF with ureteric obstruction treated with methyl-prednisolon pulse therapy (MPPT) in combination with azathioprine or penicillamine following initial insertion of ureteral stents. The medial treatment suggested was successful in 7 patients, but only moderately effective in the last 4 patients. This principle of non-operative management of RF has not been previously reported.

[Comparison of indirect immunofluorescence on Crithidia luciliae of Farr test, and immunoenzymatic methods for the screening of anti-native DNA autoantibodies]. / Comparaison de l'immunofluorescence indirecte sur Crithidia luciliae du test de Farr, et des méthodes immunoenzymatiques pour le dépistage des autoanticorps anti-ADN natif.

Anti-native DNA (ds DNA) antibodies were measured by the Farr assay, an indirect immunofluorescence on Crithidia luciliae (IIFCL) and a newly commercialised enzyme-linked immunosorbent assay (Elisa) in the sera of 270 patients: 73 negative controls, 67 patients with systemic lupus erythematosus (SLE), 73 with clinical symptoms of another connective tissue disease and 61 patients with liver diseases. The Farr assay remains a reliable method, IIFCL seems to be as specific but less sensitive and Elisa has a higher sensitivity but a lower specificity. Finally, 35 sera from the 270 patients were tested for anti-ds DNA antibodies using two other commercialised Elisa-related techniques. The results suggest little correlation between the three Elisa methods.

Impact of regular relaxation training and psychosocial working factors on neck-shoulder tension and absenteeism in hospital cleaners.

Occupational stress in hospital cleaners (n = 50), and the effect of relaxation training (n = 25, age matched, randomized), were studied by recording the electrical activity of the upper trapezius muscle at rest and during working conditions at the beginning, middle, and end of a 6-month follow-up period. A short (15-minute) relaxation program was practiced daily at the workplace to provide stress management. The amount of sick leave was counted, and the extent of depression and some psychosocial working factors were screened. Intercorrelations were found between the neck-shoulder tension, psychosocial factors, depression, and the absentee rate. The relaxation training diminished tension in the neck-shoulder region efficiently; nevertheless, the decrease in absenteeism might have been related mainly to the social support offered by the research maneuver itself.

Ulcerative colitis complicating seronegative HLA-A2-B27 rheumatoid arthritis with sacroiliitis.

The case is reported of a 50 year old man with longstanding seronegative rheumatoid arthritis who developed ulcerative colitis. The patient also had sacroiliitis and his tissue was typed as HLA-A2-B27 several years before the bowel disease began. A possible overlap between primary inflammatory bowel disease, complications to the treatment of rheumatoid arthritis with drugs, and gastrointestinal rheumatoid vasculitis is discussed.

No signs of Campylobacter jejuni/coli-related antibodies in patients with active ankylosing spondylitis.

Twenty-two patients with active ankylosing spondylitis were investigated to assess the levels of specific serum IgG, IgA and IgM titres against Campylobacter jejuni/coli before and during treatment with sulfasalazine. An enzyme-linked immunosorbent assay was used, and the results were compared with the antibody levels in 300 healthy blood donors. Three patients had elevated levels of serum anti-Campylobacter-IgA before treatment, and a two-fold decrease in the antibody titre was observed during treatment. Three patients had elevated anti-Campylobacter-IgG titres before treatment. One of these patients also had elevated anti-Campylobacter-IgA and IgM titres. Elevated IgM titres were not seen in any other patient. The results do not support the hypothesis that C. jejuni/coli plays an important role in the pathogenesis of active AS.

Ankylosing spondylitis--education, employment and invalidity.

Two hundred forty-eight patients suffering from ankylosing spondylitis (AS) completed a questionnaire about the social impact of the disease. Of the 84% who replied to the questions about employment status, 118 patients were employed full-time. The ability to manage full-time employment was reduced in 41 patients. Eighty-five patients indicated that they had changed occupation or educational status as a cause of AS; 46 of these later experienced a more progressive course, which reduced their working capacity, and 31 retired as invalids. Despite the long-term morbidity with gradual loss of functional capacity, 85% were still able to work after more than 20 years of illness. If we pay more attention to initial symptoms related to AS, delay in the diagnosis of AS could be decreased and social instability avoided by guidance in education and light occupations.

Relaxation training combined with increased physical activity lowers the psychophysiological activation in community-home boys.

Resting electrical activity (EMG) in the frontalis, temporalis, trapezius and erector trunci muscles, as well as systolic and diastolic blood pressure (BP), were measured in boys in a community home, and in controls of the same age in an ordinary school. EMG in the community-home boys was significantly higher than in controls, whereas BP did not yield any difference. The community-home boys participated in a programme consisting of relaxation training and increased physical activity for 4 months. After this intervention, EMG in all muscle groups was decreased, and the level of EMG was also lower than the values measured in controls. Systolic and diastolic BP in the community-home boys was also lowered, but the decrease was not significant as compared to the control group. In conclusion, relaxation training and/or increased physical activity is effective in decreasing the elevated activation observed in community-home boys.

Hand handicap and rheumatoid arthritis in a fish-eating society (the Faroe Islands).

In the county of Klaksvik, the Faroes, a simple hand test was used for screening all inhabitants between the ages of 40 and 74 years. With its high sensitivity and specificity it revealed a diagnostic panorama entirely different from that seen in previous investigations in other nordic countries. In Klaksvik the prevalence of hand handicap was about 40%, which is due to the high incidence of osteoarthrosis. The prevalence of rheumatoid arthritis was 1.1%. The high functional capacity and the lower occurrence of rheumatic nodules and erosions found in the patients with rheumatoid arthritis as compared with previous studies suggest that the disease takes a milder course in Klaksvik. This should support the hypothesis that RA patients benefit from a diet rich in fish.

A preliminary trial of high-dose intravenous immunoglobulin to a patient with euthyroid ophthalmopathy.

A patient with serious euthyroid infiltrative ophthalmopathy was treated with oral glucocorticoids for five years. The course of the disorder was characterized by recurrent exacerbations during attempts to reduce the dose of systemic glucocorticoids. Intravenous infusion with high-dose immunoglobulin gave immediately favourable regression of the lesion, without side-effects. Possible modes of action of high-dose immunoglobulin treatment are discussed.

HLA types in patients with rheumatoid arthritis developing leucopenia after both gold and sulphasalazine treatment.

HLA types, especially HLA-DR3, are associated with the development of toxic reactions in patients with rheumatoid arthritis after treatment with gold or D-penicillamine. In this study, after treatment with sulphasalazine, leucopenia was observed in three patients, who all had a history of leucopenia after previous gold treatment. The HLA types of these patients did not include HLA-DR3; the two patients developing mild leucopenia had HLA-DR2 and the one developing agranulocytosis had HLA-DR4.