The objective of the trial is to assess the efficacy, safety, and tolerability of BI 685509 compared with placebo on a background of local standard of care (SOC) therapy in the treatment of adult patients with early progressive dcSSc and vasculopathy
Inclusion Criteria
1. Patients must be diagnosed with diffuse cutaneous SSc (widespread skin fibrosis with skin involvement proximal to elbows and/or knees) as defined by LeRoy et al. (R17-0149).
2. SSc disease onset (defined by first non-RP symptom) must be within 5 years of Visit 1.
3. Evidence of active disease, defined as having at least one of the following:
- New onset of SSc within the last 2 years of Visit 1 OR
- New skin involvement or worsening of two new body areas within 6 months of Visit 1 OR
- New involvement or worsening of one new body are if either chest or abdomen within 6 months of Visit 1 OR
- Worsening of skin thickening within 6 months of Visit 1 OR
- At least one tendon friction rub
Exclusion Criteria
1. Any known form of pulmonary hypertension
2. Limited cutaneous SSc at screening. Other autoimmune connective tissue diseases, except for fibromyalgia, scleroderma-associated myopathy and secondary Sjogren syndrome.
3. Diffusing capacity for carbon monoxide (DLCO) (haemoglobin corrected) <40% of predicted at screening.
Study Requirements
t least 14 in-person visits to Tufts Medical Center for at least 13 months - 12 blood collections - 12 urine colections - 1 high resolution CT scan
