Demographic and clinical properties of juvenile-onset Behçet's disease: A controlled multicenter study

PubMedID: 18045733Background: Behçet's disease (BD) is a multisystemic inflammatory disorder of unknown origin. The disease usually occurs between the second and the fourth decades, whereas it is uncommon in children. Objective: In this multicenter study, we aimed to describe the demographic and clinical features along with severity in juvenile- versus adult-onset BD. Methods: Patients with initial symptoms at age 16 years or younger were considered as having juvenile-onset BD. In all, 83 patients with juvenile-onset BD (38 male and 45 female; mean age 19.6 ± 7.6 years) and 536 with adult-onset (>16 years) BD (293 male and 243 female; mean age 39.2 ± 10.1 years) who fulfilled the classification criteria of the International Study Group for BD were involved in the study. Results: Familial cases were more frequent in juvenile-onset compared with adult-onset BD (19% vs 10.3%; P = .017). The mean age of disease onset was 12.29 ± 3.54 years in juvenile-onset BD and 31.66 ± 8.71 years in adult-onset BD. Mucocutaneous lesions and articular symptoms were the most commonly observed manifestations in both groups. The frequency of disease manifestations was not different between juvenile- and adult-onset BD, except neurologic and gastrointestinal involvement, which were higher in juvenile-onset BD than adult-onset BD (P = .027 and P = .024, respectively). Oral ulcer was the most common onset manifestation of both juvenile-onset (86.74%) and adult-onset (89.55%) BD. The frequencies of onset manifestations of BD were similar, except genital ulcer, which was higher in adult-onset BD (P = .025). Limitations: Our study consisted of patients with BD mainly applying to dermatology and venerology departments. Therefore, it can be speculated that this study includes rather a milder spectrum of the disease. Conclusions: Although the clinical spectrum of juvenile-onset BD seems to be similar to adult-onset BD, the frequency of severe organ involvement was higher. Because of the higher prevalence of familial cases in juvenile-onset BD, it can be speculated that genetic factors may favor early expression of the disease with severe organ involvement. © 2008 American Academy of Dermatology, Inc

Clinical features and natural course of Behçet's disease in 661 cases: A multicentre study

PubMedID: 17711526Background: Behçet's disease (BD) is a systemic inflammatory disease with unpredictable exacerbations and remissions. The natural course of BD is not fully known. Objectives: We aimed retrospectively to determine the occurrence of the symptoms in chronological order. We also evaluated the influence of the treatment and follow-up on the clinical severity and tried to identify the factors determining severe organ involvement. Methods: Six hundred and sixty-one patients were involved in this multicentre study. The symptoms of the disease were recorded retrospectively in the time order of the manifestations in each patient. Results: Oral ulcers were the most common manifestation (100%), followed by genital ulcers (85.3%), papulopustular lesions (55.4%), erythema nodosum (44.2%), skin pathergy reaction (37.8%), and articular (33.4%) and ocular involvement (29.2%). Oral ulcers were the most common onset manifestation (88.7%). The mean ± SD duration between the onset symptom and the fulfilment of diagnostic criteria was calculated to be 4.3 ± 5.7 years. The clinical severity score was significantly increased in the noncompliant treatment group compared with the compliant group with the passage of time (P < 0.001). The frequency of ocular involvement and genital ulcers was significantly higher in patients whose disease onset was at < 40 years. Genital ulcers, ocular involvement, papulopustular lesions, thrombophlebitis and skin pathergy reaction were found to be significantly more frequent in males. Conclusions: Mucocutaneous lesions are the hallmarks of the disease, and especially oral ulcers precede other manifestations. The increase in clinical severity score is more pronounced in patients without regular treatment and follow-up. Male sex and a younger age at onset are associated with more severe disease. © 2007 The Authors