DISEASE-ACTIVITY AND AUTOANTIBODIES TO ENDOTHELIAL-CELLS IN PATIENTS WITH WEGENERS GRANULOMATOSIS

The purpose of this study was to assess the utility of antiendothelial cell antibodies (AECAs) in patients with active and inactive Wegener' s granulomatosis. We studied 32 patients with Wegener's disease (clini cal criteria and biopsy, as well as titers of antineutrophil cytoplasm ic antibodies with a cytoplasmic pattern [cANCA]) over 4 years and com pared their AECA values with those of 24 normal subjects similar in ag e and gender distribution, as well as with those of patients with chro nic glomerulonephritis with or without dialysis and of patients with s evere arteriosclerosis, We measured AECAs, cANCAs, C-reactive protein, erythrocyte sedimentation rate, proteinuria, and renal function in pa tients with active disease or in patients reactivating their disease, A time course with repeated AECAs was conducted over 27 months in 24 p atients, The AECAs were measured with an enzyme-linked immunosorbent a ssay. The specificity was verified with immunofluorescent confocal mic roscopy, which showed the AECA epitopes to be within the cytoplasm of endothelial cells. Elevated AECA titers were found in all patients wit h active disease, not all of whom had positive cANCAs. Although elevat ed AECAs were also found in some patients with inactive disease, norma l AECA values were seen only in patients with inactive disease. Patien ts with active disease entering remission showed a decrease in AECA ti ters, while patients entering a relapse increased their AECA titers. W e conclude that AECAs are present in patients with Wegener's granuloma tosis. To our knowledge, these are the first serial AECA observations. Our data suggest that AECAs are correlated with disease activity. Ant iendothelial cell antibody values in the normal range strongly support remission. These findings may be of clinical utility in distinguishin g relapse from concomitant illness. (C) 1996 by the National Kidney Fo undation, Inc.