Objective
Behçet's disease (BD) is a chronic, relapsing, inflammatory vascular disease with no pathognomonic test. Low sensitivity of the currently applied International Study Group (ISG) clinical ...diagnostic criteria led to their reassessment.
Methods
An International Team for the Revision of the International Criteria for BD (from 27 countries) submitted data from 2556 clinically diagnosed BD patients and 1163 controls with BD‐mimicking diseases or presenting at least one major BD sign. These were randomly divided into training and validation sets. Logistic regression, ‘leave‐one‐country‐out’ cross‐validation and clinical judgement were employed to develop new International Criteria for BD (ICBD) with the training data. Existing and new criteria were tested for their performance in the validation set.
Results
For the ICBD, ocular lesions, oral aphthosis and genital aphthosis are each assigned 2 points, while skin lesions, central nervous system involvement and vascular manifestations 1 point each. The pathergy test, when used, was assigned 1 point. A patient scoring ≥4 points is classified as having BD. In the training set, 93.9% sensitivity and 92.1% specificity were assessed compared with 81.2% sensitivity and 95.9% specificity for the ISG criteria. In the validation set, ICBD demonstrated an unbiased estimate of sensitivity of 94.8% (95% CI: 93.4–95.9%), considerably higher than that of the ISG criteria (85.0%). Specificity (90.5%, 95% CI: 87.9–92.8%) was lower than that of the ISG‐criteria (96.0%), yet still reasonably high. For countries with at least 90%‐of‐cases and controls having a pathergy test, adding 1 point for pathergy test increased the estimate of sensitivity from 95.5% to 98.5%, while barely reducing specificity from 92.1% to 91.6%.
Conclusion
The new proposed criteria derived from multinational data exhibits much improved sensitivity over the ISG criteria while maintaining reasonable specificity. It is proposed that the ICBD criteria to be adopted both as a guide for diagnosis and classification of BD.
To determine whether the incidence of gout is higher in 1995-1996 compared to 1977-1978.
Using the Rochester Epidemiology Project computerized medical record system, all potential cases of acute gout ...in the city of Rochester, Minnesota during the time intervals of 1977-1978 and 1995-1996 were identified. The complete medical records of all potential cases were screened and all who fulfilled the 1977 American College of Rheumatology proposed criteria for gout were included as incidence cases. Demographic data, body mass index, clinical presentation, and associated comorbid conditions were abstracted. The overall and age-gender adjusted incidence rates from the 2 cohorts were calculated and compared.
A total of 39 new cases of acute gout were identified during the 2 year interval 1977-1978 representing an age and sex-adjusted annual incidence rate of 45.0/100,000 (95% CI: 30.7, 59.3). For the interval 1995-1996, 81 cases were diagnosed, representing an annual incidence rate of 62.3/100,000 (95% CI: 48.4, 76.2). There was a greater than 2-fold increase in the rate of primary gout (i.e., no history of diuretic exposure) in the recent compared to the older time periods (p = 0.002). The incidence of secondary, diuretic related gout did not increase over time (p = 0.140).
Our results indicate that the incidence of primary gout has increased significantly over the past 20 years. While this increase might be a result of improved ascertainment of atypical gout, it may also be related to other, as yet unidentified, risk factors.
We report a case of arterial occlusive disease and digital ischaemia associated with acute parvovirus B19 infection. Treatment with intravenous epoprostanol improved her symptoms.
We report a case of arterial occlusive disease and digital ischaemia associated with acute parvovirus B19 infection. Treatment with intravenous epoprostanol improved her symptoms.
