CFHR4 and acute pancreatitis: CFHR4 encodes one of the 5 complement factor H-related proteins and is linked to aHUS, a life threatening TMA characterized by dysregulation of the alternative pathway of complement [59]. While aHUS rarely causes acute pancreatitis and the association between these two diseases remain unclear, several reports revealed an episode of acute pancreatitis preceding TMA in cases with or without TMA related mutations [60–62].