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

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Background

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.

Section snippets

Methods

Records of patients with BD from 9 medical centers in Turkey (Akdeniz, Baskent, Cukurova, Erciyes, Firat, Gazi, Inonu, Mersin, and Uludag Universities) were involved in this study.

A registration form and a detailed study questionnaire was prepared in the core center (Inonu University Medical Faculty Hospital, Malatya, Turkey) and sent to the satellite study centers. Medical records of all patients with juvenile- and adult-onset BD who were suitable to be included were cited into these forms and

Results

A total of 83 patients with juvenile-onset BD (38 male and 45 female) with a mean age of 19.6 ± 7.6 years (between 5 and 46 years) and 536 patients with adult-onset BD (293 male and 243 female) with a mean age of 39.2 ± 10.1 years (between 19 and 75 years) were enrolled. Mean age at the time of onset was 12.3 ± 3.5 years (between 1.1 and 16.0 years) in juvenile-onset BD and 31.7 ± 8.7 years (between 17.1 and 71.1 years) in patients with adult-onset BD. Family history was positive in 19% of

Discussion

Since the first article on pediatric BD by Mundy and Miller,14 several case reports and case studies have been published on this issue. Some of these publications stress that family history is more prominent, diagnosis is delayed, and disease course is more severe in pediatric patients with BD than adult counterparts, whereas other publications claim that no difference in these parameters exists.7, 8, 9, 10, 11, 12, 13, 14, 15, 16, 17, 18, 19 There is little literature comparing adult- with

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  • Cited by (126)

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    Funding sources: None.

    Conflicts of interest: None declared.

    Presented as free communication at the XII International Conference on Behçet's Disease, in Lisbon, Portugal, on September 20-23, 2006.

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