Senicapoc in Patients With Worsening Fibrotic Interstitial Lung Disease

Inclusion Criteria: * Progressive fibrotic ILD or Progressive IPF diagnosed according to ATS/ERS/JRS/ALAT guidelines at the time of diagnosis * Age \> 18 years * HRCT historically performed within 24 months * FVC \> 45 %, FEV1/FVC \> 0,7 or above LLN * Annual FVC decline of at least 5% predicted, based on at least three FVC measurements within 6-24 months before enrolment * Subject able to give informed consent. * The extent of fibrotic changes is greater than the extent of emphysema on the most recent HRCT scan * Male subjects of reproductive potential agree to use highly effective contraception/preventive exposure measures from the time of first dose of IMP during the study, and until 90 days (male) after the last dose of IMP. * Female subjects agree to use highly effective contraceptive during the study, and must show a negative pregnancy test before inclution. * Able to walk at least 150 meters during the 6MWT at screening Visit 1; * Able to read and complete the EQ-5D, SGRQ-I, K-BILD questionnaire. Exclusion Criteria: * Sickle cell disease * Any clinical condition or other condition or circumstance that, in the opinion of the investigator, may make a subject unsuitable for inclusion or unlikely or unable to complete the study or comply with study procedures and requirements. * Known hypersensitivity to any of the IMP ingredients or a history of a significant allergic reaction to any drug as determined by the investigator * A current immunosuppressive condition * Clini…