Arthritis & Rheumatism, Volume 62,
November 2010 Abstract Supplement
Abstracts of the American College of
Rheumatology/Association of Rheumatology Health Professionals
Annual Scientific Meeting
Atlanta, Georgia November 6-11, 2010.
The Disease Presentation and Outcome in Juvenile Behcet's Syndrome.
Seyahi3, Emire, Ozdogan3, Huri, Ozyazgan2, Yilmaz, Ugurlu3, Serdal, Ozguler1, Yesim, Hatemi3, Gulen, Yazici3, Hasan
To assess clinical characteristics and outcome among patients with juvenile onset Behcet's syndrome (BS) all attending a single dedicated center.
We reviewed the charts of around 7000 patients registered between July 1977 and December 2009 at our multidisciplinary BS outpatient clinic. We surveyed patients who fulfilled the International Study Group (ISG) criteria for BS and who were 16 or younger at their initial visit. All these patients were called back within a survey period of 3 months. Demographic and clinical characteristics at initial and final visit were determined.
There were 166 patients (86 boys, 80 girls). The mean age at first visit was 14.2 ± 1.9 years, the mean age at appearance of first symptom 10.9 ± 3.1 years, and the mean age at ISG fulfillment 13.1 ± 2.6 years. At the time of first visit, patients had oral ulcers (100%; 166/166), genital ulcers (68%; 113/166), pathergy positivity (63%; 104/166), papulopustular lesions (57%; 94/166), erythema nodosum (42%; 69/166), arthritis (20%; 33/166), eye disease (48%; 80/166), vascular disease (13%; 22/166) and neurological disease (8%; 13/166). The clinical manifestations at first visit such as genital ulcer, papulopustular lesions, erythema nodosum and arthritis were less frequent compared to the adult population (1). The dural sinus thrombi type of neurological involvement was the most common type of involvement (85%; 11/13). Familial history of BS was present in 41 (25%) patients. Information on the onset of puberty was available only in 94 patients. The onset was prepubertal in 39 and postpubertal in the remaining 55. While erythema nodosum was more common among those with prepubertal onset (24/39 vs 16/55), genital ulcer was more common among those with postpubertal onset (18/39 vs 44/55). A total of 13 (8%) were lost to follow-up after a single visit. Six (4%) (all males) had died. The median follow-up time in the remaining was median 10 years . Causes of death in 6 males were pulmonary artery aneurysms (n=2), hepatic failure due to Budd-Chiari syndrome (n=1), suicide (n=2) and pneumonia (n=1). At the end of follow-up 18 patients (22.5%) had lost useful vision (bilateral: 8, unilateral: 10). The visual acuity in either eye was between 0.61.0 in 44 (56%) and between 0.50.1 in the remaining 18 (22.5%). Two patients (all males) with neurological involvement had severe neurological deficit (hemiplegia and optic atrophy. As seen adults, skin-mucosa lesions and joint involvement stopped occurring during the follow-up. Among 77 patients (50 M/27 F) in whom a final evaluation was available after a median of 9  years, we observed that oral ulcer (90%) and papulopustular lesions (55%) were more frequent compared to genital ulcers (18%), erythema nodosum (29%) and arthritis (14%).
Pediatric cases made up around 0.2% of all Behçet patients. As in the adult, BS runs a severe course among the boys considering overall mortality in addition to the vascular and neurological involvement.
To cite this abstract, please use the following information:
Seyahi, Emire, Ozdogan, Huri, Ozyazgan, Yilmaz, Ugurlu, Serdal, Ozguler, Yesim, Hatemi, Gulen, et al; The Disease Presentation and Outcome in Juvenile Behcet's Syndrome. [abstract]. Arthritis Rheum 2010;62 Suppl 10 :1697