ANTIENDOTHELIAL CELL ANTIBODIES IN SCLERO DERMA SYSTEMATICA AND RAYNAUDS-DISEASE

Using ELISA on fixed endothelial hybridoma (EAhy.926) cells we investi gated the occurrence of antiendothelial antibodies (AEA) in the sera f rom patients with scleroderma systematica (SS) (n = 70) and Raynaud's disease (RD) (n = 19). The mean IgG and IgA-AEA levels were significan tly higher in the SS patients than RD patients (p < 0.001) and control s (p < 0.001). We have detected circulating IgG-AEA in 64.2% of patien ts with SS,. 35.2% of SS sera were positive for IgA-AEA. In patients w ith RD, the frequency of AEA corresponded to that of the random popula tion sample. We have found a high incidence of Raynaud's phenomenon, m yositis, telangiectasia and marked digital ischaemia (digital ischemic pulp ulcers, digital scares, osteolysis and autoamputation) among AEA positive patients with SS. AEA-positive patients were characterized b y high extent and severity of Raynaud's phenomenon and higher avenge n ailfold capillary microscopy scores. No correlations were found betwee n AEA and different clinical or laboratory parameters, including the t ype of scleroderma (diffuse and limited), the presence of anti Scl-70 and anticentromere antibodies and the clinical features of SS (lungs, kidneys and heart involve ment, esophageal dysfunction, calcinosis, Sj ogren's syndrome). There was no significant correlation between the AE A level and patient age, extent of skin involvement (skin score). Thus , in SS, AEA is associated with a peripheral vasculopathy and represen t a useful marker for the diagnosis of endothelial dysfunction.