Chronic intestinal inflammation can occur in people with cystic fibrosis (CF), although no treatment is recommended. Although Lumacaftor/Ivacaftor is well-known for modulating the faulty cystic fibrosis transmembrane conductance regulator (CFTR) protein in the lungs, no evidence on its influence on CF intestinal problems are available. As a result, researchers studied the progression of intestinal inflammation following the beginning of Lumacaftor/Ivacaftor in CF adolescents (median of follow-up: 336 days [IQR: 278;435]).

When Lumacaftor/Ivacaftor was started, median fecal calprotectin concentrations dropped considerably (102 g/g [IQR: 69–210]) compared to the baseline (713 g/g (IQR:148–852), P = 0.001). This study demonstrated for the first time that Lumacaftor/Ivacaftor therapy reduces CF-related intestinal inflammation.