Senicapoc in Patients With Progressive Fibrotic ILD (Interstitial Lung Disease) and IPF (Idiopathic Pulmonary Fibrosis) to Prevent Progression.
This study will investigate whether the senicapoc drug can prevent the scarring from worsening in interstitial lung disease. Researchers will compare Senicapoc to a placebo (a look-alike substance that contains no drug) to see if Senicapoc works to prevent lung function worsening. Participants will be asked to take 3 tablets a day for 26 weeks. Within this period, doctors will follow the participants, ask for experience of adverse events, check lung function and organ status, and participants will need to fill out quality-of-life questionnaires. A total of 5 visits are required, at initiation, after4, 13, 26 and 52 weeks. The final visit will occur 52 weeks after initiation and consist of a normal visit in the outpatient clinic where the doctor asks for relevant information regarding the period after end of administration of the study drug.
• 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.