No abstract

Objective. To identify and recombine a protein of the human dermal microvascular endothelial cell (HD-MEC) that specifically reacts with anti-endothelial cell antibody (AECA) in the serum of patients with Behçet's disease (BD), and to evaluate the usefulness of this protein in BD.Methods. The proteomics technique, with 2-dimensional gel electrophoresis and matrix-assisted laser desorption ionization؊time-of-flight (MALDI-TOF) mass spectrometry, was used to identify and recombine HDMEC antigen. Western blotting and enzyme-linked immunosorbent assay (ELISA) of recombinant protein isolated by gene cloning were performed on serum from healthy controls, patients with BD, and patients with other rheumatic diseases (rheumatoid arthritis, systemic lupus erythematosus, and Wegener's granulomatosis).Results. Eighteen of 40 BD patients had serum IgM antibody to HDMEC antigen. The purified protein that reacted with AECA in BD patient sera was found to be ␣-enolase by 2-dimensional gel electrophoresis followed by immunoblotting and MALDI-TOF mass spectrometry. Recombinant ␣-enolase protein was isolated and refined by gene cloning. On Western blots, AECApositive IgM from the sera of patients with active BD reacted strongly with recombinant human ␣-enolase. BD patient sera positive for anti-␣-enolase did not react with human ␥-enolase. On dot-blotting, reactivity to human ␣-enolase was detected only in the IgM-positive group. Fifteen of the 18 AECA-positive sera that were positive for the HDMEC antigen showed reactivity to recombinant ␣-enolase IgM antibody by ELISA.Conclusion. The ␣-enolase protein is the target protein of serum AECA in BD patients. This is the first report of the presence of IgM antibodies to ␣-enolase in endothelial cells from the serum of BD patients. Although further studies relating this protein to the pathogenesis of BD will be necessary, ␣-enolase and its antibody may prove useful in the development of new diagnostic and treatment modalities in BD.

Behçet's disease is a chronic, relapsing, idiopathic, multi-system condition characterized by mucocutaneous, ocular, gastrointestinal, articular, vascular, urogenital, and neurologic involvement. Usually the onset occurs between 20 and 30 years of age; it is very uncommon in children. We analyzed 40 cases of childhood-onset Behçet's disease. The male:female ratio was 0.67. The most frequent major sign was oral ulceration, appearing in all patients. Other major signs were genital ulcers (33 patients), skin lesions (29), and ocular lesions (11). The average time interval between the initial oral ulceration and the second major manifestation was 8.8 years. After the second major manifestation, the third and fourth features rapidly developed within one to two years. The most frequent minor sign was arthritis, occurring in 11 of 40 patients. Intestinal, neurologic, and renal involvement was also present. From our results, we concluded that oral ulceration, which is the most common initial manifestation, should not be neglected in children, since it may signal Behçet's disease.

The prevalence of Behcet's disease is the highest in the East Asian and the Mediterranean countries. Behcet's disease is also distributed in the Asian countries, but the nationwide survey has not been performed in Korea yet. The Korean Study Group for Behcet's Disease, founded in 1999, conducted a multicenter, retrospective survey on epidemiologic and clinical features of the patients with Behcet's disease from 20 hospitals around the nation from 1997 to 1999. Of 3,497 patients, 1,527 were classified into complete or incomplete type of Behcet's disease according to the revised Shimizu's classification. The sex ratio was 1:1.75 with the female predominance. Geographical distribution showed the highest frequency in Seoul (38.5%). Clinically, 98.8% had oral ulcers, 83.2% had genital ulcers, 84.3% had skin lesions and 50.9% had ocular lesions. As for the minor clinical manifestations, articular symptoms were the most frequent. The pathergy test showed positive in 15.4% of patients and revealed a higher positive rate in males (20.2%) than in females (12.7%). In conclusion, we performed the first multicenter study on Behcet's disease in Korea and revealed the female predominance, higher frequency of ocular lesions, and lower positivity of pathergy test in the patients.

Behçet's disease is a multisystem disorder that affects mainly young adults. Conflicting reports of the effects of pregnancy on the course of Behçet's disease have been reported. We studied 27 pregnant women with Behçet's disease using questionnaires, clinical examination, and laboratory tests. The condition of 18 patients worsened (66.7%) and 9 patients (33.3%) improved during pregnancy. In the deteriorated group, clinical exacerbation of Behçet's disease occurred most commonly during the first trimester (77.8%). They were primarily of the suspected or mucocutaneous type, while in the improved group, the majority of patients were of the incomplete and complete type, or the arthritic and ocular type. Of interest was the fact that all patients in the improved group showed exacerbations related to menstruation or postpartum, which indicated that progesterone may be the major hormone influencing the course of Behçet's disease.