Clinical features of Behçet's disease in children: An international collaborative study of 86 cases,☆☆,

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Abstract

Objectives: The objective of this study was to characterize the clinical picture of Behçet's disease (BD) in children. Study design: A questionnaire was completed by five BD specialists from Turkey, France, Iran, or Saudi Arabia. We first reviewed 86 cases retrospectively with a specially designed computerized database and then selected 65 who met the criteria of the International Study Group for BD, which include buccal aphthosis plus at least two among recurrent genital aphthosis, eye lesions, skin lesions, and positive pathergy test. The remaining 21 patients, who had features suggestive of BD but did not fulfill the international criteria, were analyzed separately and then compared with the other 65 patients. Results: BD affected boys and girls equally. The clinical picture frequently included mucocutaneous lesions. Uveitis was less frequent than in adults but carried a poor prognosis, especially in male patients (p < 0.001). The mortality rate (3%) was related to large vessel involvement. Familial cases were particularly frequent (15%). Erythema nodosum and skin hypersensitivity were common in Turkish patients, whereas neuro-BD was more frequent in French and Saudi Arabian patients. Patients who did not fulfill the international criteria had significantly less genital aphthosis (p < 0.01), less skin lesions or hypersensitivity (p < 0.01), and less uveitis (p < 0.01). Conclusion: BD in children is similar to BD in adults. The high frequency of familial cases calls for further investigation of the immunogenetic factors that may favor early expression of the disease. (J Pediatr 1998;132:721-5.)

Section snippets

Patients and Methods

A questionnaire was mailed to five specialists of BD in four different countries including Turkey (two centers: Istanbul and Ankara), Saudi Arabia, Iran, and France. The files of all patients with BD before the age of 16 years were reviewed retrospectively. The questionnaire included 286 items divided into 10 groups including identification, familial history, mucocutaneous, neurologic, psychiatric, ocular, digestive, pulmonary, cardiac, and nephrourologic features. The chronology of these

Epidemiology

Sixty-five patients were reviewed: 33 boys, 32 girls (sex ratio 1.03) from Turkey (37), France (13), Saudi Arabia, (8) and Iran (7). Most of them were white and of Turkish or West European descent. The mean age at onset of the disease was 8.4 years (0 to 16 years) (Fig. 1, A), and the mean age at diagnosis was 13 years (3 to 16 years) (Fig. 1, B).

. A, Age at onset of Behçet's disease in 65 children. B, Age at diagnosis of Behçet's disease in 65 children.

The follow-up was at least 5 years for 32%

Discussion

Although most cases of BD were observed in late childhood (mean 13 years, median 15 years), three patients had the disease during the first year of life. However, we observed no neonatal cases of BD caused by an affected mother.11, 12 Another unexpected finding was the relatively high rate of familial cases (15%) compared with adult BD (2%).3 This is of particular interest in determining the respective roles of genetic and environmental factors that may influence the expression of BD.

The

Acknowledgements

We are grateful to Paul Mandelbröjt for support, to Marie Thérèse Jimeno and Bernard Guisiano for statistics, and to Samia Zouraghi and Elisabeth Vistue for secretarial assistance.

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    From the Department of Pediatrics, Hôpital Nord and Hôpital de la Timone, Marseilles, France; Department of Medicine (Division of Rheumatology) Cerrahpasa Medical Faculty, University of Istanbul, Turkey; Department of Pediatrics, King Faisal Specialist Hospital and Research Center, Riyadh, Kingdom of Saudi Arabia; the Rheumatology Research Center, Shariaty Hospital, Tehran, Iran; and the Department of Pediatric Nephrology and Rheumatology, Hacettepe University, Ankara, Turkey.

    ☆☆

    Reprint requests: Isabelle Koné-Paut, MD, Service de Pédiatrie, Hôpital Nord, chemin des Bourrelys, 13915 Marseilles, France.

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