Evidence for a “Pathogenic Triumvirate” in Congenital Hepatic Fibrosis in Autosomal Recessive Polycystic Kidney Disease

Figure 3

A “pathogenic triumvirate” in congenital hepatic fibrosis (CHF) in autosomal recessive polycystic kidney disease (ARPKD). Published data suggest that cyst growth, fibrosis, and inflammation drive CHF/ARPKD (solid purple arrows). We propose that relationships exist between cyst growth, fibrosis, and inflammation which drive progression of CHF/ARPKD (black, double-headed arrows with broken lines). Targeting a single pathway which drives each of the triumvirate members, or targeting multiple members concurrently, may provide better therapeutic strategies than if targeting any one member in isolation.