[Comparison of the accuracy of diagnostic criteria of behcet's disease in children]

Different criteria for Behcet's diagnosis reveal that there isn't still consensus about the definition of this syndrome. Although a comprehensive study about the accuracy, the sensitivity and specificity of seven different types of diagnostic criteria for Behcet's disease in children, Ras not been performed we planned this study to compare these criteria in the Iranian children with Behcet's disease. Two random samples were selected from the patients less than 16 years of age referring to the Behcet's clinic of rheumatology research center. 177 children had definite diagnosis of Behcet's disease after being visited by the three professors of rheumatology experienced in Behcet's syndrome. Control group were children suspected to have Behcet's disease but it was ruled out. Sensitivity, specificity and accuracy of each of the Behcet's criteria were calculated. Mean age in Behcet's children was 13.6 years and in control group was 12.8 years. In the patients group female to male ratio was 1.1 and in the control group it was 1.3. Sensitivity of different criteria was as follow: Manson and Bames 55.1%, 0' Duffy 60.7%, International criteria 71.9%, Japan criteria 90.4%, Dilsen criteria 79.8%, Iran criteria 93.8%, and Iranian classification tree 94.4%. Specificity of the criteria was as following; M and B 99.5%, O Duffy 99.5%, International 100%, Japan 96.2%, Dilsen 94.6%, Iran 96.2%, classification tree 96.2%. Although some of the researchers believe that current diagnostic criteria of Behcet's diseases due to establishment on the adults clinical data, is not appropriate for children; our study revealed that the current seven Adult BD criteria are reliable and accurate in children too

Scleroderma in pediatric age group: Report of 25 cases

Scleroderma is a multisystem autoimmune connective tissue disease, characterized by hardening of the skin. We had 25 patients in the Iranian Pediatric Unit of Rheumatology over 10 years. There was female predominance [F/M = 4/1, 80% female, 20% male]. The age of onest was from 6 months to 16 years, the mean age being 10 years. The symptoms and signs were: nonpitting edema in 16%, hard skin in 80% sclerosis of distal limbs in 72%, facial in 20%, atrophy, and hypopigmentation or hyperpigmentation in 40%,telangiectasis [15%], subcutaneous calcification [10%], Raynaud's phenomenon in 60%, digital ulceration [16%], muscle pain and weakness, arthralgia [30%]. Rarer manifestantion included, dysphagia [16%], dyspnea [8%], cardiovascular disease, pericarditis, cardiomegaly, CHF [16%]. Laboratory abnormalities included: anemia [80%], positive FAN A. > 40 [88%] ESR > 60 [40%] CRP+++ [40%]. Skin biopsy documented increase in the collagen content of dermis in 75%