A Study to Test How Well BI 3000202 is Tolerated by People With Type 1 Interferonopathies

This study is open to adults with selected type 1 interferonopathies. People can join the study if they have Aicardi-Goutières syndrome (AGS), Coatomer subunit alpha (COPA) syndrome, Familial chilblain lupus (FCL), or another type 1 interferonopathy with a specific gene mutation. The purpose of this study is to find out how BI 3000202 is tolerated in people with selected type 1 interferonopathies. Participants take a lower dose of BI 3000202 as tablets for 4 weeks. Afterwards, they take a higher dose of BI 3000202 as tablets for 36 weeks. They may continue with the study treatment until every participant has completed 40 weeks of treatment (about 9 months). The participants may also continue their regular treatment for their condition during the study. During this study, participants visit the study site 13 times or more, depending on when they start their participation. The doctors check the health of the participants and note any health problems that could have been caused by BI 3000202.

NCT Number	NCT06878365
Lead Sponsor	Boehringer Ingelheim
Conditions	Type 1 Interferonopathies
Enrollment	16 participants
Start Date	2025-07-29
Primary Completion	2026-12-06 (estimated)
Study Completion	2026-12-07 (estimated)
Updated on ClinicalTrials.gov	2026-07-07