Autoimmune Hepatitis/Primary Sclerosing Cholangitis Overlap Syndrome Associated With Minimal Change Disease and Tubulointerstitial Nephritis

ABSTRACT

Autoimmune hepatitis/primary sclerosing cholangitis overlap syndrome is rare and incompletely defined. Its association with renal disease is less frequently reported. Systemic disorders such as systemic lupus erythematosus, immunoglobulin G4-related disease, light chain disorders, amyloidosis, and pediatric autoimmune polyendocrine syndromes may cause concurrent hepatic and renal involvement; however, renal disease with liver pathology in the absence of systemic illness is exceedingly uncommon. We describe a case of autoimmune hepatitis/primary sclerosing cholangitis overlap complicated by nephrotic syndrome due to minimal change disease with tubulointerstitial involvement. No systemic disease or drug-induced cause was identified. Renal function fully recovered with treatment while liver disease persisted.