ABC008-IBM-202 is an open-label, multicenter study to evaluate the safety and efficacy of long-term administration of ulviprubart (ABC008) in subjects with IBM who have completed either Study ABC008-IBM-101 or Study ABC008-IBM-201. Subjects may be enrolled in this study if they meet study eligibility criteria and: * Have completed the Part 2 (Multiple Ascending Dose \[MAD\]) End of-Treatment (EOT) Visit in Study ABC008-IBM-101; subjects who continued further on into Part 3 of the study (MAD Extension) prior to enrolling in this study are also eligible; OR * Have completed the Week 80 Follow-up Visit in Study ABC008-IBM-201.
ABC008-IBM-202 is an open-label, multicenter study to evaluate the safety and efficacy of long-term administration of ulviprubart (ABC008) in subjects with IBM who have completed either Study ABC008-IBM-101 or Study ABC008-IBM-201. Subjects may be enrolled in this study if they meet study eligibility criteria and: * Have completed the Part 2 (Multiple Ascending Dose \[MAD\]) End of-Treatment (EOT) Visit in Study ABC008-IBM-101; subjects who continued further on into Part 3 of the study (MAD Extension) prior to enrolling in this study are also eligible; OR * Have completed the Week 80 Follow-up Visit in Study ABC008-IBM-201. Subjects will enter this study following their initial study such that dosing continues every eight weeks (Q8W) between the last dose in the initial study and the first dose in this long-term extension (LTE) Study IBM-202. Study subjects will continue to receive any ongoing concomitant medications from the initial study. Subjects will be required to sign an informed consent form before undertaking any study-specific procedures or assessments. Screening is intended to be done at the final visit of the initial study, which will coincide with the Baseline (Day 1) Visit for this study; if this is not possible, the Baseline (Day 1) Visit for this study, including screening, may be conducted at a separate visit (provided it occurs within the visit window defined in Appendix 1 of the protocol). Subsequent study visits will occur every eight weeks until EOT at Week 156, or withdrawal from the study.
Study Type
INTERVENTIONAL
Allocation
NA
Purpose
TREATMENT
Masking
NONE
Enrollment
270
All eligible subjects, regardless of treatment assignment or dose level in their initial study, will be administered ulviprubart at a dose of 2.0 mg/kg via subcutaneous (SC) injection Q8W.
Neuromuscular Research Center
Phoenix, Arizona, United States
University of California Irvine Medical Center (UCIMC) - Amyotrophic Lateral Sclerosis (ALS) and Neuromuscular Center
Irvine, California, United States
Keck Hospital of USC
Los Angeles, California, United States
Yale School of Medicine
New Haven, Connecticut, United States
Neuromuscular Diagnostic Center - Massachusetts General Hospital
Boston, Massachusetts, United States
Brigham and Womens Hospital
Boston, Massachusetts, United States
Hospital for Special Surgery
New York, New York, United States
Duke Neurological Disorders Clinic
Durham, North Carolina, United States
University Hospitals Cleveland Medical Center
Cleveland, Ohio, United States
Ohio State University Wexner Medical Center
Columbus, Ohio, United States
...and 11 more locations
Primary Endpoint
The primary endpoint for the study will be the incidence, type, and severity of treatment-emergent adverse events (TEAEs). Safety as assessed by the incidence, type and severity of Treatment Emergent Adverse Events (TEAEs)
Time frame: From Baseline (Day 1) through study completion, an average of 156 weeks.]
Treatment Emergent Serious Adverse Events (TEASAEs)
Incidence, type, and severity of treatment-emergent serious adverse events (TESAEs)
Time frame: From Baseline (Day 1) through study completion, an average of 156 weeks.]
Treatment Emergent Adverse Events (TEAEs) onset within 24 hours of Study Medication Administration.
Incidence of TEAEs leading to study medication or study discontinuation
Time frame: From Baseline (Day 1) through study completion, an average of 156 weeks.]
Adverse Events of Special Interest (AESI)
Incidence of Adverse events of special interest (AESIs)
Time frame: From Baseline (Day 1) through study completion, an average of 156 weeks.]
Change from baseline standard laboratory parameters (Hematology)
Incidence of clinically significant changes in standard laboratory parameters (Hematology)
Time frame: From Baseline (Day 1) through study completion, an average of 156 weeks.]
Change from baseline in standard laboratory parameters (Chemistry)
Incidence of clinically significant changes from baseline in standard laboratory parameters (Chemistry)
Time frame: From Baseline (Day 1) through study completion, an average of 156 weeks.]
Change from baseline in standard laboratory parameters (Coagulation)
Incidence of clinically significant changes from baseline standard laboratory parameters (Coagulation)
Time frame: From Baseline (Day 1) through study completion, an average of 156 weeks.]
Inclusion Body Myositis Functional Rating Scale (IBMFRS)
Mean change from Baseline (Day 1) in IBMFRS over the duration of the study
Time frame: From Baseline (Day 1) through study completion, an average of 156 weeks.]
Manual Muscle Test 12 (MMT 12)
Mean change from Baseline (Day 1) in MMT 12 over the duration of the study
Time frame: From Baseline (Day 1) through study completion, an average of 156 weeks.]
Change from baseline in standard vital signs (respiratory rate)
Incidence of clinically significant changes in vital signs (respiratory rate)
Time frame: From Baseline (Day 1) through study completion, an average of 156 weeks.]
Change from baseline in standard vital signs blood pressure
Incidence of clinically significant changes in standard vital signs blood pressure (Systolic and diastolic) blood pressure)
Time frame: From Baseline (Day 1) through study completion, an average of 156 weeks.]
Change from baseline in standard vital signs (temperature)
Incidence of clinically significant changes in standard vital signs (temperature)
Time frame: From Baseline (Day 1) through study completion, an average of 156 weeks.]
Change from baseline in standard vital signs (pulse rate)
Incidence of clinically significant changes in standard vital signs (pulse rate)
Time frame: From Baseline (Day 1) through study completion, an average of 156 weeks.]
Counts of absolute and KLRG1+ lymphocytes by flow cytometry.
Blood samples will be collected for immunophenotyping. Counts of absolute and KLRG1+ lymphocytes and other lymphocyte subsets will be performed by flow cytometry.
Time frame: From Baseline (Day 1) through study completion, an average of 156 weeks.]
Presence and titer of antidrug antibodies (ADA)
The incidence and titers of ADAs to ulviprubart will be evaluated in blood samples
Time frame: From Baseline (Day 1) through study completion, an average of 156 weeks.]
Serum concentration of ulviprubart
Individual and mean serum concentration time profiles will be presented graphically using nominal time points.
Time frame: From Baseline (Day 1) through study completion, an average of 156 weeks.]
This platform is for informational purposes only and does not constitute medical advice. Always consult a qualified healthcare professional.