Non-cirrhotic Portal Fibrosis with Coexisting Non-lupus Full House Nephropathy: An Uncommon Dual Organ Involvement

Non-cirrhotic portal fibrosis (NCPF) is an uncommon cause of portal hypertension; its coexistence with non-lupus full-house nephropathy (NLFHN) is exceedingly rare. We report a 27-year-old man with anasarca, nephrotic-range proteinuria, and hematemesis. Evaluation showed esophageal varices, splenomegaly, preserved liver enzymes, ANA negativity, and isolated Scl-70 positivity. Kidney biopsy revealed diffuse endocapillary hypercellularity with wire loop lesions and full-house immunofluorescence, consistent with Class IV immune complex glomerulonephritis. Liver biopsy confirmed NCPF with obliterative venopathy. Treatment with corticosteroids and mycophenolate mofetil led to marked proteinuria reduction and stable hepatic status over six months, highlighting this rare renal–hepatic overlap and need for recognition.