Objective: By functioning as a heat-shock protein (HSP), alpha-enolase has an important role in the pathophysiology of multivariant vasculitis. Kawasaki disease (KD) is a type of vasculitis occurring primarily in children. The role of alpha-enolase in KD was assessed by measuring anti-alpha-enolase antibody (Ab) titres in patients with KD and the usefulness of anti-alpha-enolase Ab as a diagnostic tool in atypical KD patients was evaluated.
Methods: Anti-alpha-enolase Ab titres were measured by using an enzyme-linked immunosorbent assay (ELISA) in seven normal control patients, nine febrile control patients and 14 KD patients (10 typical KD, four atypical KD). A standard deviation (SD) of 3 above the mean of the normal control group was considered to be positive reactivity. Western blotting using recombinant human alpha-enolase was performed in four KD patients and three normal controls.
Results: With the positive reactivity limited to +3 SD over the mean (>0.6), 10 out of 14 patients (71%) were positive at the acute onset and 12 out of 14 patients (85.7%) were positive before discharge. In total, 12 out of 14 patients (85.7%) were positive either at acute onset or before discharge. All four atypical KD patients showed positive reactivity. Specific positive bands against recombinant human alpha-enolase were detected by western blotting in all four KD patients, but no reactivity was seen in three patients with normal controls.
Conclusion: This is the first study to demonstrate that autoantibodies against the alpha-enolase are present in the sera of KD patients. We suggest that anti-alpha-enolase Ab should be a good candidate for a diagnostic tool in atypical KD.