Resolution of portal hypertension in a patient with cystic fibrosis after treatment with CFTR modulator: A case report.

Abstract

Portal hypertension (pH) secondary to cystic fibrosis liver disease (CFLD) is the fourth most common cause for mortality (after respiratory/cardiorespiratory, transplant-related, and cancer-related) in adults with cystic fibrosis (CF) and more often occurs in the absence of cirrhosis (i.e. non-cirrhotic pH, NCPH). Here, we describe a patient with NCPH secondary to CFLD, with resolution of pH after starting a cystic fibrosis transmembrane conductance regulator (CFTR) modulator. As demonstrated in this patient, CFTR modulators may provide extra-pulmonary benefits including reversal of NCPH. Long-term use of CFTR modulators could potentially result in reductions in mortality from pH and need for future liver or combined lung-liver transplantation in patients with CFLD.