Progressive systemic sclerosis
Conditions
Interventions
IMP administration etc.
BI 685509 will be up-titrated as tolerated
Sponsors
Adachi Sayaka
Eligibility
Sex/Gender
All
Inclusion criteria
Inclusion criteria: - Signed and dated written informed consent in accordance with ICH-GCP and local legislation prior to admission to the trial. - Male or female patients aged 18 years at time of consent (or above legal age). - Patients must fulfil the 2013 American College of Rheumatology/European Alliance of Associations for Rheumatology (ACR/EULAR) classification criteria for SSc. - Patients must be diagnosed with limited or diffuse cutaneous SSc (widespread skin fibrosis with skin involvement proximal to elbows and/or knees) as defined by LeRoy et al. - Diffuse cutaneous SSc disease onset (defined by first non-RP symptom) must be within 7 years of Visit 1. Limited cutaneous SSc onset must be within 2 years of Visit 1. - Evidence of active disease.
Exclusion criteria
Exclusion criteria: - Any known form of pulmonary hypertension. - Other autoimmune connective tissue diseases, except for fibromyalgia, scleroderma-associated myopathy and secondary Sjogren syndrome. - Diffusing capacity for carbon monoxide (DLCO) (haemoglobin corrected) 4 x upper limit of normal (ULN), or ALP > 2 x ULN and gamma-glutamyl transferase (GGT) > 3 x ULN at Screening. - Known, severe gastric antral telangiectasias (watermelon stomach). - Any history of bronchial artery embolization or massive hemoptysis. (Massive hemoptysis is defined as acute bleeding >240 mL in a 24-hour period or recurrent bleeding >100 mL/day over consecutive days). - Active hemoptysis or pulmonary hemorrhage, including events managed by bronchial artery embolization. - Systolic blood pressure <100 mm Hg or known history of moderate or severe symptomatic orthostatic dysregulation as judged by the Investigator before start of trial treatment. - Sitting heart rate (HR) <50 beats per minute (BPM) at the Screening Visit. - Left ventricular ejection fraction <40% prior to screening. - A marked baseline prolongation of QT/QTc interval.
Design outcomes
Primary
| Measure | Time frame |
|---|---|
| FVC(Forced vital capacity) | — |
Secondary
| Measure | Time frame |
|---|---|
| mRSS (Modified Rodnan Skin Score) | — |
Countries
Argentina, Australia, Austria, Belgium, Brazil, Canada, Chile, China, Czechia, Denmark, Finland, France, Germany, Greece, India, Israel, Italy, Japan, Malaysia, Mexico, Netherlands, New Zealand, Norway, Philippines, Poland, Portugal, Republic of Korea, Roumania, Singapore, Spain, United States
Contacts
Public ContactNobuko Yamada
Boehringer Ingelheim
Outcome results
None listed