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.


Pediatric Behet's Disease, PED-BD: An International Cohort Study of 110 Patients. One-Year Follow-Up Data.

Kone-Paut11,  Isabelle, Bello11,  Martha Darce, Shahram15,  Farahd, Gattorno7,  Marco, Cimaz10,  Rolando, Ozen2,  Seza, Hofer14,  Michael

Bicêtre University Hospital, Clinical Research Unit, Le Kremlin-Bicêtre, France
Pediatric Rheumatology, A. Meyer Institut Florence, Florence, Italy
Pediatric Rheumatology, Bicêtre University Hospital, Le Kremlin-Bicêtre, France
Pediatric Rheumatology, Robert Debré University Hospital, Paris, France
Pediatric Rheumatology, Toulouse University Hospital, Toulouse, France
Pediatric Rheumatology, Vaudois University Hospital, Lausanne, Switzerland
Rheumatology Research Center, Shariati Hospital, Tehran, Islamic Republic of Iran
Rhumatology, Childrens Hospital, University of Tuebingen, Tübingen, Germany
Hacettepe University, Department of Pediatrics, Ankara, Turkey
Hospital Alexandria Hospital, Alexandria, Egypt
IBN Rochid University Hospital, Casablanca, Morocco
Istanbul University-Ophtalmology, Istambul, Turkey
Pediatric Clinic, Blegdamsvej, Copenhagen, Denmark
Pediatric Rheumatology G. Gaslini Scientific Institut, Genoa, Italy
Pediatric Rheumatology King Fayçal Hospital, Riyadh, Saudi Arabia
Pediatric Rheumatology Unit, Sant Joan de Déu University Hospital, Esplugues de Llobregat, Spain

Aim of the Study:

To set up an algorithm for definition of pediatric Behçet's disease (PED-BD) based on international cohort of children suspected with BD

Patients and Methods:

An international expert committee has defined the inclusion criteria as follows: Recurrent oral aphthosis (ROA) associated with at least one of following: genital ulceration (GU), erythema nodosum, folliculitis, pustulous/acneiform lesions, positive pathergy test, uveitis, venous/arterial thrombosis, family history of BD. Onset of disease is before the 16th birthday, disease duration is <= 3 years, minimum future follow up duration is 4 years and to obtain informed consent. Clinical data are updated every year. Accordingly, the included patients are classified by the expert committee into 3 groups: definite PED-BD, probable PED-BD and no PED-BD. Statistical analysis are performed at the end to compare the 3 groups of patients. Centers specializing in PED-BD have been called to collaborate and document their patients into a single database, (available online).

Results:

In January 2010, 110 patients (56M/54F) from 16 centres of 11 countries have been included. Mean age at first symptom: 8.1 y (median 8.2,). Mean age at BD suspicion: 11.8 y, (median 14.4y). At inclusion 38 % of them had only 1 symptom associated with ROA, 31% had 2 and 31% had at least 3. 106 first evaluations have been done. Chronology of symptoms at presentation: 59 patients with 1 symptom (ROA: 45, and fever:5 being the most frequent)), 26 with 2 symptoms (OA + GU: 6), 14 with 3 and 3 with 4.

Figure 1. Clinical signs (percentages) at first visit, according to their gender, in 106 PED-BD patients.

93% were receiving treatment: colchicine 59%, steroids 58%, and azathioprine 14%. Fifty-seven patients underwent the first year evaluation and 36 had no new symptom, 12 had one, and 9 had 2. The expert committee has examined 48 files and classified 30 as definite and 18 as probable. Among our patients classified as definite, 26 (87%) fulfilled the ISG criteria. 17/18 classified as probable did not meet the international criteria (p< 0.001)].

Table. List of symptoms in addition to oral aphthosis in patients classified as definite and probable by the committee of experts.

 Confirmed n=30Probable n=18
Genital ulceration*217
Skin lesions**223
Uveitis166
Arthralgia/arthritis117
Gastro-intestinal126
Neurological***123
Vascular71
Urological10
Fever136
Pulmonary30
Cardiac30
Family history34
Significant values:
*p=0.03;**p=0.0001;***p=0.09

Conclusion:

The expert committee has classified the majority of patients in the BD group although they presented with few symptoms independently from BD classification criteria.

To cite this abstract, please use the following information:
Kone-Paut, Isabelle, Bello, Martha Darce, Shahram, Farahd, Gattorno, Marco, Cimaz, Rolando, Ozen, Seza, et al; Pediatric Behet's Disease, PED-BD: An International Cohort Study of 110 Patients. One-Year Follow-Up Data. [abstract]. Arthritis Rheum 2010;62 Suppl 10 :1305
DOI: 10.1002/art.29071

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