AUTOIMMUNE INTERSTITIAL NEPHRITIS AND HEPATITIS IN POLYGLANDULAR AUTOIMMUNE SYNDROME

A 6-year-old female with polyglandular autoimmune syndrome type I, chr onic active hepatitis, and renal failure is described. The renal biops y demonstrated advanced tubulointerstitial disease with antibodies dir ected against tubular basement membranes, The patient's serum containe d circulating antibodies directed against both renal and hepatic paren chyma. Renal disease culminating in renal failure and anti-tubular bas ement membrane disease have not been previously reported in associatio n with polyglandular autoimmune disease. We describe for the first tim e a patient with polyglandular autoimmune syndrome, chronic active hep atitis, circulating antibodies directed against both renal and hepatic parenchyma, and primary tubulointerstitial disease culminating in ren al failure.