Pathogenicity of Antiendothelial Cell Autoantibodies

Publisher Summary Although the pathogenicity of anti-EC antibodies (AECA) remains uncertain, the likelihood of such effects was first denoted by the observation that the autoantibody levels fluctuate with disease activity in patients with systemic lupus erythematosis (SLE), Wegener granulomatosis (WG), and Kawasaki syndrome (KS). The pattern of AECA reactivity in a given SLE patient has been reported to change as the disease entered different phases. AECAs have also been commonly detected in patients with systemic vasculitis, and their levels reported to parallel disease activity in WG, even more tightly than anti-neutrophil cytoplasm antibodies (ANCA). In keeping with these preliminary clinical observations, the time of sample collection might be important in the detection of AECAs in KS. the AECA test can identify particular subsets of SSc, vasculitides or inflammatory myopathies with related evolution. The detection of AECAs in patients with primary Raynaud's phenomenon, limited systemic sclerosis (SSc) or diffuse SSc may help to determine the long-term prognosis of the disease. A significant trend for AECAs to increase with disease severity across these three groups of patients indicates that the AECA test can identify subsets of SSc with differing prognoses.