Çocukluk Çağı Behçet Hastalığı Vakalarının Retrospektif Değerlendirilmesi

Behçet disease is a multisystemic vasculitis characterized by oral ulcers accompanied with skin and mucosa manifestations. Turkey is one of the regions where disease is more common. There is not enough demographic data in literature about Behçet disease so in our country there is need for pediatric Behçet case reports. In this study data of patients, who were diagnosed as Behçet disease and treated in Hacettepe University Ihsan Dogramaci Pediatric Hospital Pediatric Nephrology and Rheumatology Departments between 01th January 1980 and 01th January 2013, collected by retrospective search. Thirty four patients (18 male, 16 female) were included in the study. We used criteria of the International Study Group for the diagnosis of Behçet disease. Any patient with recurrent oral ulcers and 2 of other criterion (genital ulcers, other skin lesions, uveitis, pathergy) was included the study. Medial age at first admission was 11,18±3,34 and most frequent first complaint was oral apthae (97%). In 15% of patients there was family history. Those patients with vasculitis or any important organ involvement were treated with pulse steroid and immunosuppressive drugs and sometimes when it is required with anticoagulants. Our results show that although Behçet disease is rare in childhood than adulthood clinical signs are similar and there is correlation between family history and frequency of disease which may sign that there are some genetic factors in disease development.Behçet hastalığı tekrarlayan oral ülserler, cilt ve mukoza bulguları ile seyreden multisistemik bir vaskülittir. Türkiye hastalığın en sık olduğu bölgelerden biridir. Çocukluk çağında daha nadir görülen bu hastalıkla ilgili yeterli sayıda demografik bilimsel çalışma olmadığından ülkemizdeki çocukluk çağı Behçet hastalığı olgularının değerlendirilmesi önemlidir. Çalışmamızda Hacettepe Üniversitesi İhsan Doğramacı Çocuk Hastanesi Pediatrik Nefroloji ve Romatoloji Bilim Dalları'nda 01.01.1980 ile 01.01.2013 tarihleri arasında Behçet hastalığı tanısıyla takip edilen hastaların dosyaları geriye dönük olarak incelenerek veri toplanmıştır. Çalışmaya dahil edilen 34 hastanın 18'i erkek ve 16'sı kızdı. Tanı koyarken Uluslararası Behçet Hastalığı Çalışma Grubu'nun tanı kriterleri kullanılmıştı. Buna göre tekrarlayan oral ülserlerine ek olarak 2 diğer kriteri (genital ülserler, diğer cilt lezyonları, üveit, paterji) bulunan hastalar çalışmaya dahil edildi. İlk başvuru sırasındaki ortalama yaş 11,18±3,34'tü. İlk başvuruda en sık şikayet %97 sıklıkla oral afttı. Hastaların %15'inde ailede Behçet hastalığı hikayesi vardı. Vasküler tutulum veya ciddi organ tutulumu olan hastalar yüksek doz intravenöz steroid ve immunsupresif ile birlikte gerektiğinde antikoagülan tedavi ile izlenmişlerdi. Sonuçlarımız Behçet hastalığının çocuklarda daha nadir görülmesine rağmen bulgularının erişkinlerle benzerlik gösterdiğini ancak aile hikayesinin sıklığının genetik faktörlerin varlığına işaret ettiğini göstermiştir

Behçet’s disease in children

Aim: In this study, it was aimed to summarize the demographics, and clinical and laboratory findings of children who were diagnosed as having Behçet’s disease, and also to determine the efficacy, duration, and adverse effects of the treatments. Material and Methods: The records of 34 patients who were diagnosed according to the International Behçet Study Group criteria between 1980 and 2013 in the Department of Pediatric Nephrology and Rheumatology, Hacettepe University Faculty of Medicine, were retrospectively reviewed and all demographic and clinical features were recorded. In the light of these data, the sex and age distribution, clinical and laboratory findings, most commonly preferred treatment approaches, efficacy of treatments, and adverse effects during treatment were analyzed. Results: Of the 34 children with Behçet’s disease, 18 (53%) were male and 16 (47%) were female, and the mean age was 11.18±3.34 years. There was no significant difference in age distribution of the male and female patients (p<0.05). In 97% (n=33) of the patients, the first symptom was recurrent oral aphthae. In order of frequency, the other mucocutaneous findings were pseudofoliculitis and pustular lesions (82%), genital ulcers (62%), and pathergy positivity (50%). System involvements in order of frequency were as follows: joint findings (38%), ocular findings (35%), vascular involvement (32%), neurologic involvement (18%), gastrointestinal involvement (5.8%), and pulmonary involvement (5.8%). Colchicine was the most commonly preferred drug (88%). Steroids were added to treatment in patients with skin involvement. Azathioprine was added in patients with uveitis. Anticoagulant therapy, cyclophosphamide, and anti-tumor necrosis factor-alpha were added in patients with vascular involvement. In patients with gastrointestinal system involvement, sulfasalazine was added to treatment. Diarrhea was the most common adverse effect in patients who used colchicine. In the patients who used steroid treatment, gastrointestinal symptoms such as unintentional weight gain, acne, and agitation were observed (17%). One patient who received interferon treatment had symptoms of depression and agitation. Conclusion: The aim of this study was to review the general characteristics of pediatric patients with Behçet’s disease and to emphasize the importance of early diagnosis and correct treatment in terms of mortality and morbidity.PubMedWoSScopu