Epstein-Barr virus genome-positive tubulointerstitial nephritis associated with Kawasaki disease-like coronary aneurysms

Abstract

A patient with recurrent renal failure due to massive interstitial nephritis caused by Leu 3a + 3b-positive T-cell infiltration and associated with multiple thromboembolic attacks is reported. He died of gastrointestinal bleeding after treatment with anticancer agents. At autopsy, diffuse necrosis of the bilateral kidneys was noted as well as giant coronary aneurysms filled with thrombus that resembled those seen in Kawasaki disease and multiple old myocardial infarcts were also present. Among the various Epstein-Barr virus (EBV)-specific antibodies, the titers anti-viral capsid antigen (VCA) and anti-early antigen (EBEA) IgG antibody were always very high in contrast to the relatively low titers of anti-EB nuclear antigen (EBNA) antibodies. DNA extracted from kidney tissue obtained at autopsy was analyzed by Southern blot hybridization after the amplification of EBV-specific DNA by the polymerase chain reaction. In situ hybridization of kidney tissue obtained at biopsy was also performed using an enzyme-linked probe derived from the EBV-encoded RNA 1 (EBER1) gene. As a result, the EBV genome was found both at autopsy and in the biopsy tissue, which clearly revealed EBER1 in the interstitial cells. Taking account of the progressive ST-T changes of the electrocardiograms which were normal early in his course, multiple myocardial infarction associating multiple giant aneurysms probably occurred during this disease process. Thus, it could be concluded that chronic active EBV infection contributed massive interstitial nephritis mediated by the activation of Leu 3a + 3b-positive T cells.