SECONDARY FOCAL SCLEROSING GLOMERULOSCLEROSIS ASSOCIATED WITH CONGENITAL HEPATIC FIBROSIS
Dr Shyamalee Crocker1
1North Canberra Hospital, Bruce, Australia
Background: Secondary focal sclerosing glomerulosclerosis is a condition commonly associated with reflux nephropathy, obesity, viruses, medications and is often due to another primary renal condition. Congenital hepatic fibrosis is a rare autosomal recessive condition often associated with both autosomal dominant and recessive polycystic kidney disease and ciliopathies. It is a developmental disorder of the porto-biliary system characterised by the defective modelling of the embryonic ductal plate. This condition is not known to be associated with other renal pathologies.
Case Report: A 42 year-old man was referred due to an elevated serum creatinine of 162umol/L with eGFR of 44ml/min in the context of non-alcoholic cirrhotic liver disease, massive splenomegaly and thrombocytopenia with platelets of 62 x10⁹L. He was diagnosed with a congenital liver condition from birth but had scant details. The patient was normotensive with non-nephrotic range proteinuria with urine albumin-creatinine ratio of 42.7mg/mmol with no red cell casts on microscopy. Anti-nuclear antibody (ANA) was positive with 1:160 titre with nucleolar pattern, but viral hepatitis screen and the rest of the autoimmune testing was negative. Renal tract ultrasound demonstrated bilateral cortical atrophy with reduced corticomedullary differentiation. He was seen by a hepatologist who commenced him on a beta-blocker for variceal prophylaxis and arranged endoscopy. He had no known family history of renal or liver disease. Renal biopsy was attended which demonstrated secondary focal sclerosing glomerulosclerosis (FSGS) with negative immunofluorescence. He was commenced on low-dose renin-angiotensin blockade with reduction in proteinuria to a urine albumin-creatinine ratio of 18.9mg/mmol and relatively static serum creatinine of 153umol/L.
Conclusions: Secondary focal sclerosing glomerulosclerosis (FSGS) may have further associations that are currently unknown with further research required.