EPSTEIN-BARR-VIRUS GENOME-POSITIVE TUBULOINTERSTITIAL NEPHRITIS ASSOCIATED WITH KAWASAKI DISEASE-LIKE CORONARY ANEURYSMS

A patient with recurrent renal failure due to massive interstitial nep hritis caused by Leu 3a+3b-positive T-cell infiltration and associated with multiple thromboembolic attacks is reported. He died of gastroin testinal bleeding after treatment with anticancer agents. At autopsy, diffuse necrosis of the bilateral kidneys was noted as well as giant c oronary aneurysms filled with thrombus that resembled those seen in Ka wasaki disease and multiple old myocardial infarcts were also present. Among the various Epstein-Barr virus (EBV)-specific antibodies, the t iters anti-viral capsid antigen (VCA) and anti-early antigen (EBEA) Ig G antibody were always very high in contrast to the relatively low tit ers of anti-EB nuclear antigen (EBNA) antibodies. DNA extracted from k idney tissue obtained at autopsy was analyzed by Southern blot hybridi zation after the amplification of EBV-specific DNA by the polymerase c hain reaction. In situ hybridization of kidney tissue obtained at biop sy was also performed using an enzyme-linked probe derived from the EB V-encoded RNA 1 (EBER1) gene. As a result, the EBV genome was found bo th at autopsy and in the biopsy tissue, which clearly revealed EBER1 i n the interstitial cells. Taking account of the progressive ST-T chang es of the electrocardiograms which were normal early in his course, mu ltiple myocardial infarction associating multiple giant aneurysms prob ably occurred during this disease process. Thus, it could be concluded that chronic active EBV infection contributed massive interstitial ne phritis mediated by the activation of Leu 3a+3b-positive T cells.