Minimal change nephropathy presenting in a patient with primary sclerosing cholangitis

The association of primary sclerosing cholangitis and renal disease is not frequent, and is limited to a few reported cases of immune complex glomerulonephritis. We report the case of a 34-year-old patient with sclerosing cholangitis diagnosed 5 years earlier, with well preserved liver function and no clinical manifestations of cholestasis, who developed minimal change nephropathy. During the nephrotic phase of the disease, the peripheral blood lymphocyte count was normal, with a relative increase in percent CD4+ and an increase in the CD4+: CD8+ ratio. CD4+ cells showed immunoactivation. The HLA-DR expression on T-cells was 59%, and 16.5% of CD3+ cells were CD25+. A course of prednisone therapy induced long-lasting remission of the nephrotic syndrome. Peripheral blood lymphocyte count and subtyping were normal 7 months after prednisone withdrawal. We conclude that primary sclerosing cholangitis can be associated with minimal change nephropathy; underlying cell-mediated immunity may be the common pathogenic mechanism.