Scleroderma, Systemic Sclerosis (SSc)
Conditions
Keywords
Scleroderma, Systemic Sclerosis (SSc), Tibulizumab, Cutaneous, Interstitial lung disease (ILD), Autoimmune diseases, Immune system diseases, Systemic Sclerosis interstitial lung disease (SSC-ILD), Skin Diseases
Brief summary
The study is a Phase 2, multi-center, randomized, double-blind, placebo-controlled study to evaluate the effects of tibulizumab over 24 weeks (Period 1) in adult participants with systemic sclerosis, followed by an open-label extension period where all active participants will receive tibulizumab and will be evaluated for an additional 28 weeks (Period 2)
Interventions
BIOLOGICALTibulizumab
Anti BAFF/IL-17 antibody
OTHERPlacebo
Placebo (inactive)
Sponsors
Zura Bio Inc
Study design
Allocation
RANDOMIZED
Intervention model
PARALLEL
Primary purpose
TREATMENT
Masking
TRIPLE (Subject, Investigator, Outcomes Assessor)
Eligibility
Sex/Gender
ALL
Age
18 Years to 75 Years
Healthy volunteers
No
Inclusion criteria
* Male or female, 18 to 75 years of age * Body mass index between 18.0 and 38.0 kg/m² * Fulfills classification criteria for SSc according to ACR/EULAR 2013 criteria * Has diffuse cutaneous SSc, defined as mRSS \>0 over at least one skin area proximal to the elbows and/or knees, in addition to acral fibrosis * Has had SSc (defined as the first non-Raynaud's phenomenon (RP) symptom or sign attributed to SSc) for ≤7 years * mRSS ≥15 and ≤45 at screening. Additional requirements for participants ≥2 years to ≤7 years from SSc onset and RNA Polymerase III antibody positive * FVC \>50% predicted * Diffusing capacity of the lungs for carbon monoxide (DLCO) ≥40% predicted (corrected for hemoglobin)
Exclusion criteria
* Has any of the following complications: * Left ventricular failure * Pulmonary arterial hypertension * Renal crisis within previous 6 months * Gastrointestinal dysmotility within previous 3 months * Digital ischemia with gangrene, amputation, or unscheduled hospitalization within previous 3 months * Current rheumatic disease other than SSc that could interfere with assessment of SSc * Lung disease requiring continuous oxygen therapy * Evidence or suspicion of active or latent tuberculosis * Active Crohn's Disease or ulcerative colitis
Design outcomes
Primary
| Measure | Time frame | Description |
|---|---|---|
| [Period 1] Change from baseline in modified Rodnan Skin Score (mRSS) at 24 weeks | Week 24 | The mRSS assesses skin thickness at 17 different cutaneous sites across the body using a scale from 0 (normal skin) to 3 (severe thickening), with a total score ranging from 0 (no thickening) to 51 (severe thickening in all 17 areas) |
| [Period 2] Safety and tolerability of tibulizumab | Week 52 | Assessed by the incidence of all treatment-emergent adverse events (TEAEs), as well as changes from baseline in vital signs, electrocardiogram (ECG) parameters, and laboratory results |
Secondary
| Measure | Time frame | Description |
|---|---|---|
| [Period 1] Change from baseline in quantitative interstitial lung disease (QILD) obtained with high-resolution quantitative tomography (HRCT) in the whole lung in participants with SSc-interstitial lung disease (ILD) | Week 24 | HRCT measures the extent of interstitial lung disease by quantifying the amount of lung fibrosis, ground-glass opacity, and honeycomb lung, which are used to calculate the QILD score (scores can range from 0 to 100%, with higher scores indicating greater disease extent) |
| [Period 1] Change from baseline in forced vital capacity (FVC) in participants with SSc-ILD | Week 24 | FVC is the amount of air that can be forcibly exhaled after the deepest possible breath |
| [Period 1] Change from baseline in Health Assessment Questionnaire Disability Index (HAQ-DI) | Week 24 | HAQ-DI evaluates patients' self-assessed degree of disability across eight categories, with scores ranging from 0 to 3, where 3 indicates the worst degree of disability |
| [Period 1] Safety and tolerability of tibulizumab | Week 24 | Assessed by the incidence of all TEAEs, as well as changes from baseline in vital signs, ECG parameters, and laboratory results |
| [Period 2] Change from baseline in mRSS | Week 52 | The mRSS assesses skin thickness at 17 different cutaneous sites across the body using a scale from 0 (normal skin) to 3 (severe thickening), with a total score ranging from 0 (no thickening) to 51 (severe thickening in all 17 areas) |
| [Period 2] Change from baseline in QILD obtained with HRCT in the whole lung in participants with SSc-ILD | Week 52 | HRCT measures the extent of interstitial lung disease by quantifying the amount of lung fibrosis, ground-glass opacity, and honeycomb lung, which are used to calculate the QILD score (scores can range from 0 to 100%, with higher scores indicating greater disease extent) |
Countries
Argentina, Chile, Hungary, Mexico, Poland, Romania, Serbia, Spain, United Kingdom, United States
Contacts
STUDY_DIRECTORStudy Director
Zura Bio Inc
Outcome results
None listed