A Proposal of a New Tool for the Assessment of Damage in Behçet Syndrome Uveitis: Cerrahpasa Ocular Damage Grading System.

INTRODUCTION/OBJECTIVE: There is currently no tool available to assess the severity of damage in uveitis due to Behçet's syndrome (BS). In this preliminary study, we developed a new grading system to evaluate ocular damage and assessed it in a prospective cohort. METHODS: A specialist in BS uveitis (YO) developed a grading system for ocular damage with five grades based on the extent of damage in the posterior segment. YO trained a senior and general ophthalmologist with sample fundus images. BS patients who had undergone color fundus photography during their routine visits in attack-free periods were included in the study. The color fundus photos of this prospective cohort were evaluated blindly by YO and his trainees using the new grading tool. Inter and intra-observer agreement between the graders were assessed by Cohen's kappa analysis. The evaluation of YO was considered as the gold standard. RESULTS: One hundred eighty-five eyes of 108 (29 F/79 M) patients with BS uveitis were graded for damage by two investigators. Their mean age was 38,58 years and their median ocular disease duration was 13 years. The gold standard and the two investigators exhibited substantial concordance with the ocular damage grading system. The inter- and intra-observer agreement were also almost perfect. CONCLUSION: The newly developed ocular damage grading system enables the standardization of damage severity in BS uveitis. It is imperative to conduct internal and external validations across diverse cohorts. Furthermore, future studies should investigate its correlation with other multimodal imaging methods such as fluorescein angiography and optical coherence tomography.

Assessment of deep venous thrombosis in the lower extremity in Behçet's syndrome: MR venography versus Doppler ultrasonography.

Lower extremity venous thrombosis (DVT) is the most common vascular manifestation of Behçet's syndrome (BS). Currently, Doppler ultrasonography (USG) is the most commonly preferred imaging modality in the diagnosis and follow-up of patients with acute and chronic DVT. Magnetic resonance (MR) venography, a quick and a non-invasive imaging modality, is successfully used to detect DVT in various settings. We had been unaware of studies with MR venography in BS. The aim of this study is to compare the diagnostic value of true fast imaging with steady-state precession magnetic resonance (True-FISP MR) venography and Doppler USG in the assessment of chronic DVT among patients with BS. 28 BS patients with chronic lower extremity DVT were studied. Common femoral (CFV) and femoral vein (FV) on both right and left sides were examined for the presence of thrombosis, recanalisation, collaterals and reflux. There are findings of chronic DVT in all Doppler USG images of 28 patients (45 of 56 FV and 35 of 56 CFV), while MR venography detects chronic thrombotic changes in 26/28 (93%) patients (43 of 52 FV and 28 of 52 CFV). Collateral veins are detected in 19 patients (19/28) with MR venography, whereas they are present in only 7 (7/28) with USG (P = 0.003). Furthermore, patients with severe post-thrombotic syndrome are more likely to have collateral formation on the MR compared to those without (12/14 vs 7/14; P = 0.043). Among patients with BS, MR venography might be an alternative or additional method to detect chronic thrombosis in the lower extremities.

Economic impact of juvenile idiopathic arthritis and familial Mediterranean fever.

The aim of the study was to determine the economical impact of juvenile idiopathic arthritis (JIA) and familial Mediterranean fever (FMF) in Turkey. A total of 100 patients (69 F/31 M) with JIA and 100 with FMF (68 F/32 F) who were consecutively seen in the outpatient clinic of the pediatric rheumatology department at Cerrahpasa Medical School between August 2008 and January 2009 were studied. Cost data were collected through a questionnaire filled out by the parents. The mean age (JIA: 11 ± 5 years; FMF:12 ± 4 years) and mean disease duration (JIA:5 ± 3 years; FMF: 4 ± 3 years) of the patients were similar. JIA patients were assigned to 5 subtypes (polyarticular: n = 45, oligoarticular: n = 30, systemic onset: n = 13, psoriatic: n = 6, and enthesopathy-related JIA: n = 6). Forty-nine percent of the patients with JIA were treated with anti-TNF drugs and 61% with DMARDs. All patients with FMF were using colchicine. The total annual cost of JIA (3,994 ± 4,101) was considerably higher than that of FMF (162 ± 77) (P < 0.001). Medication fee was the major determinant of total costs in both diseases constituting 85% in JIA and 39% in FMF. Among the subtypes of JIA, total annual costs were the highest among patients with polyarticular type (6,045 ± 4,078). Medications especially anti-TNF drugs were the major contributor among all determinants of costs in JIA. The low costs of health care system and prominent changes in the health care policies for the last 5 years in Turkey might have played role in our findings.