Summary:Behufs disease is characterized by orogenital ulccrations and ocular lesions, Other features include arthritis, thrombophlebitis, neurological abnormalities and skin lesions. The disease is characterized by a relapsing inflammatory process of unknown aetiology. Lipoprolcin(a) is an LDL-likc particle with a large glycoprotein called apolipoprotein(a) attached to its npolipoprotcin B moiety through one or more clisulphidc bonds. Apolipoprotein(a) is related to plasminogcn from which the cn/yme plasmin, that hydrolyscs fibrin blood clots, is released by tissue plasininogcn activators. The unique structural features of Lp(a) give it the potential for atherogenie and thrombogenic activities. In the present study 35% of patients with /ic j //(.'t'/\s disease were shown to have higher Lp(a) concentrations than the cut-off point (0.30 g/l) for atherosclerosis. Plasma Lp(a) concentrations in the remission period were also found 1ο be lower than during the active period in the same patients (23% decreased). Lp(a) showed significant correlations with acute phase rcaclanls such as crythrocyte sedimentation rate, polymorphonuclcar leukocytes and polymoφhonuclear leukocyte elastase activity. Therefore, it was concluded that the fluclations of plasma Lp(a) levels with the activity of disease may be a contributing risk factor in the development of (hrombogcnic complications in patients with Belief $ disease.In conclusion, we suggest that plasma Lp(a) concentrations be determined for patients with Belter* disease, and that patients with high Lp(a) levels be kept under close controls especially during the active period of the disease, and taken into remission as soon as possible.