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.

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.

Levamisole in a double-blind study: no effect on warts.

Levamisole is reported to restore delayed hypersensitivity, when this is depressed. On the assumption that multiple warts, resistant to local treatment, are indicative of some immune deficiency, we chose levamisole for the treatment of warts. The dose, 150 mg daily in 3 days every second week, was given to 49 patients who had common warts and to 50 patients who had condyloma (venereal warts). After 6 weeks of double-blind study, most of the patients underwent open levamisole treatment. However, the levamisole group failed to show enhanced wart regression compared with the control group. No inter-group difference could be detected as regards change in delayed hypersensitivity after levamisole treatment, when measured by the mantoux response.