Clinical Research Directory

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 * Clinically significant abnormalities detected on ECG of either rhythm or conduction, * Moderate to severe hepatic impairment (Child-Pugh B or C); and/or abnormal LFT at screening, * Clinical laboratory test suggestive of cholestasis with total serum bile acid levels \> 3xULN. * Abnormal renal function, defined as eGFT \> 30 ml/kg * History of malignancy within the past 5 years * Previous participation in a clinical study with IMP for fibrotic disease within the last 6 months. * Concurrent participation in another interventional drug, device, or biological investigational research study, or use of an investigational agent within 5 half-lives of the agent * Lower respiratory tract infection requiring treatment within 4 weeks prior to screening and/or during the screening period. * History of lung volume reduction surgery or lung transplant. * Diagnosis of severe pulmonary hypertension * Unstable cardiovascular, pulmonary (other than IPF), or other disease within 6 months prior to screening or during the screening period * Use of any of the following therapies within 4 weeks prior to screening and during the screening period, or planned during the study: warfarin, imatinib, ambrisentan, azathioprine, cyclophosphamide, cyclosporine A, bosentan, methotrexate, sildenafil (except for occasional use), prednisone at steady dose \> 10 mg/day or equivalent. * Current alcohol or substance abuse in the opinion of the investigator.