Medical use of cystic fibrosis transmembrane conductance regulator (CFTR) modulators

A method of preventing and/or treating a disease involving endothelial and/or epithelial barrier dysfunctions, the method comprising administering a pharmaceutical composition comprising a CFTR modulator as an active ingredient, wherein the disease does not comprise cystic fibrosis;

• wherein the CFTR modulator is a CFTR potentiator a CFTR corrector, or a CFTR amplifier,
• wherein the CFTR potentiator is a compound selected from Ivacaftor, GLPG2451, GLPG1837, QBW251, PTI-808, FDL176 or a deuterated compound or pharmaceutically acceptable salt thereof;
• wherein the CFTR corrector is a compound selected from Lumacaftor, Tezacaftor, Elexacaltor, Bamocaftor, Bamocaftor potassium, Posenacaftor, Cavosonstat, GLPG2222, GLPG2737, or a deuterated compound or pharmaceutically acceptable salI thereof;
• and
• wherein the OFTR amplifier is a compound selected from Nesolicaftor and PTI-CH, or a deuterated compound or pharmaceutically acceptable salI thereof.

A method of preventing and/or treating a disease involving endothelial and/or epithelial barrier dysfunctions, the method comprising administering the components of a kit of parts comprising i) a CFTR modulator and ii) a further CFTR modulator wherein the disease does not comprise cystic fibrosis;

• wherein the CFTR modulator is a CFTR potentiator, a CFTR corrector, or a CFTR amplifier,
• wherein the CFTR potentiator is a compound selected from Ivacaftor, GLPG2451, GLPG1837, QBW251, PTI-808, FDL176 or a deuterated compound or pharmaceutically acceptable salt thereof;
• wherein the CFTR corrector is a compound selected from Lumacaftor, Tezacaftor, Elexacaltor, Bamocaftor, Bamocaftor potassium, Posenacaflor, Cavosonstat, GLPG2222, GLPG2737, or a deuterated compound or pharmaceutically acceptable salI thereof;
• and
• wherein the CFTR amplifier is a compound selected from Nesolicaftor and PTI-CH, or a deulerated compound or pharmaceutically acceptable salt thereof.