Behcet's disease in Iran: Analysis of 7641 cases.

Objective: To analyze Behcet's Disease (BD) in Iran, from 1975 to 2018, and compare to 35 large/small reports from other countries.Methods: Patients from all over Iran, when suspected, were sent to the BD Unit. The diagnosis was done by expert opinion. All data were recorded in the BD registry (updated in each follow-up). The data are given in percentage with 95% confidence Intervals.Results: The mean age at onset was 25.6 years. Standard deviation (SD) was 9.8. The mean disease duration was 11.7 years (SD: 8.9). Males were 55.8% (54.7-56.9), Females 44.2% (43.1-45.3), Oral Aphthosis (OA) 97.5% (97.1-97.9), genital aphthosis (GA) 64.4% (63.3-65.5), skin lesions 62.2% (61.1-63.3), ocular lesions 55.6% (54.5-56.7), Joint Manifestations 38.1% (37.0-39.2), Gastrointestinal 6.8% (6.2-7.4), Vascular 8.9% (8.3-9.5), neurological (central-peripheral) 3.9% (3.5-4.3), epididymitis 4.6% (4.1-5.1). Lab tests were positive pathergy test 50.4% (49.3-51.5), elevated ESR 51.1% (50.0-52.2), abnormal urinalysis 13.4% (12.6-14.2). The International Study Group (ISG, 1990) criteria and the International Criteria for Behcet's Disease (ICBD, 2014) had respectively a sensitivity of 76.2% (75.2-77.2) and 96.6% (96.2-97.0). The specificity was 99.3% (99.1-99.5) and 97.3% (96.9-97.7). The accuracy was 86.4% (85.8-87.0) and 96.9% (96.6-97.2).Conclusion: The most frequent manifestations were OA, GA, skin manifestations, and ocular manifestations.

Retinopathy in newly-diagnosed systemic lupus erythematosus: should we screen for ocular involvement?

BACKGROUND: Ocular manifestations are common in systemic lupus erythematosus (SLE). Retinopathy has previously been linked to disease severity and might have a significant impact on the patient's quality of life and has also been associated with a poor prognosis in SLE. This study aimed to determine the prevalence of retinopathy among patients who are newly diagnosed with SLE. METHODS: In a cross-sectional study, patients diagnosed with SLE at a tertiary referral clinic were assessed for inclusion between March 2016 and March 2017. Patients who had received treatment for SLE at any time were excluded, as well as patients with hypertension, diabetes mellitus, and coagulopathy. Clinical findings and laboratory test results were recorded, and patients were examined by an ophthalmologist for evidence of retinal pathologies. SLE disease activity index was also calculated for all patients. RESULTS: With 114 patients included in the final analysis, we found a prevalence of 15.8% for retinopathy among newly-diagnosed SLE patients. Cotton-wool spots were the most common finding (78%). Patients with retinopathy had significantly lower hemoglobin levels, C3 and C4 concentrations, and higher ANA and Anti-dsDNA levels. Also, patients with retinopathy had a significantly higher SLE DAI score. CONCLUSIONS: We found a relatively high rate of retinopathy in SLE patients at the time of their initial diagnosis. Our findings suggest that retinopathy is an early manifestation of the disease. Ophthalmologic screening might be considered for SLE patients at the time of diagnosis, especially for those with severe disease. We also encourage researchers to further evaluate the correlation between retinopathy and disease activity, and the prognosis of ocular involvement.