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Open Label Extension Study of Brentuximab Vedotin in Early dcSSc

Phase 2
Recruiting
Conditions
Diffuse Cutaneous Systemic Sclerosis
Interventions
Registration Number
NCT05149768
Lead Sponsor
Lawson Health Research Institute
Brief Summary

The purpose of this study is to assess safety and efficacy of Brentuximab vedotin, a CD30-directed antibody-drug conjugate, in patients with active diffuse cutaneous systemic sclerosis (dcSSc) who relapsed after discontinuation of Brentuximab vedotin.

Detailed Description

Systemic sclerosis (SSc, Scleroderma) is a multisystem autoimmune disease characterized by widespread vascular injury and progressive fibrosis of the skin and internal organs. Internal organ involvement results in increased mortality of SSc patients. There is no effective treatment for the majority of patients with early active diffuse scleroderma (diffuse cutaneous systemic sclerosis; dcSSc). It's possible to reverse immune inflammation and reduce the probability of irreversible fibrosis early in the disease course via significant immune modulation. The preliminary results of the Phase II study of Brentuximab vedotin (Protocol BV201708) in SSc demonstrated the short-term safety and benefits of this treatment as many participants already achieved the primary endpoint at 24 weeks. This study is proposed as an extension of the ongoing protocol for up to 48 weeks to make the treatment available for SSc patients who have significantly improved on Brentuximab vedotin, but relapsed after discontinuation of the treatment. Similar to the ongoing Phase II study, the Health Assessment Questionnaire Disability Index (HAQ-DI), patient and physician global scores, inflammatory markers (ESR, CRP), and combined response index in SSc (CRISS) and changes in CD30-stained cells on skin biopsies with IHC will all be exploratory outcomes.

Recruitment & Eligibility

Status
RECRUITING
Sex
All
Target Recruitment
11
Inclusion Criteria
  1. Patients with diffuse cutaneous systemic sclerosis enrolled in the Phase II Adcetris study (BV201708) at St. Joseph's Health centre, aged 18 years or older, and:
  2. Worsening mRSS of ≥ 4 points as compared to mRSS score at the end of treatment visit (week 48) in the initial study (BV201708).
  3. Able to give informed consent.
Exclusion Criteria
  1. Poor pulmonary function (FVC<40% and/or DLCO<30%).

  2. Pregnancy, breast feeding or child bearing potential without practicing highly effective contraception (and partners for men in the study).

  3. Clinically significant pulmonary hypertension requiring drug therapy.

  4. Clinically significant cardiac disease.

  5. Chronic or ongoing active infectious disease requiring systemic treatment.

  6. Seropositivity for human immunodeficiency virus (HIV).

  7. Active tuberculosis (TB) infection.

  8. Active viral infection with viral replication of hepatitis B or C virus.

  9. Significant concurrent, uncontrolled medical condition including, but not limited to, renal, hepatic, pancreatic, hematological, gastrointestinal, endocrine, pulmonary, neurological, cerebral or psychiatric disease; and cancer.

  10. Peripheral neuropathy at screening Grade 2 or higher.

  11. Known or suspected hypersensitivity to components of the treatment

  12. Patients known or suspected of not being able to comply with a study protocol (e.g. due to alcoholism, drug dependency or psychological disorder)

  13. Any of the following laboratory abnormalities at screening:

    • Absolute neutrophils count <2.0 x 109/L
    • Hemoglobin <85 g/L
    • Platelet count < 100 x 109/L
    • AST/SGOT or ALT/SGPT >2.0 UNL
  14. Participation in another clinical trial within six weeks before randomization in this study, with the exception of continuation from the initial study BV201708.

  15. Use of rituximab within the previous 4 months.

  16. Immunization with a live/ attenuated vaccine less than 4 weeks prior to the baseline visit.

  17. Current or history of progressive multifocal leukoencephalopathy (PML).

Study & Design

Study Type
INTERVENTIONAL
Study Design
SINGLE_GROUP
Arm && Interventions
GroupInterventionDescription
Administration of Brentuximab vedotinBrentuximab vedotinMaximum duration of treatment: 48 weeks Maximum dose allowed: 0.6 mg/kg Route of administration: intravenous
Primary Outcome Measures
NameTimeMethod
Change in skin thickness measured by modified Rodnan Skin Score48 weeks

Skin improvement is defined as the mean mRSS decrease of ≥8 points

modified Rodnan Skin Score (mRSS): is a standard outcome measure for skin disease in SSc and calculated by measuring skin thickness in 17 different body sites (each site scored 0-3, with a total possible additive score of 51). A higher skin score (or a higher "skin thickness") and progression of this score, is predictive of internal organ involvement and mortality. While a lower or improving (lessening) score is associated with favorable outcomes, including better survival.

Secondary Outcome Measures
NameTimeMethod
Change in Scleroderma Health Assessment Questionnaire (SHAQ)12, 24, 36, and 48 weeks

The SHAQ combines the disability and pain scales of the HAQ (HAQ-DI), with five scleroderma-specific Visual Analogue Scales for: digital ulcers, Raynaud's phenomenon, gastrointestinal (GI) symptoms, lung symptoms, and overall disease severity.

The HAQ-DI yields a score of 0-3, that indicates the extent of the respondent's functional limitations.

Each VAS score is scaled from 0 to 3, with 0 being no symptoms and 3 being the worst possible symptom severity.

A composite VAS score is not created nor are the individual VAS scores incorporated into the HAQ DI score. Typically, each VAS score is reported individually. There is a proposed way to obtain a combined score obtained by pooling the 8 domains of the HAQ DI and the 5 VASs; however, this approach has not yet been widely accepted.

This is a patient reported outcome.

Change in Forced Vital Capacity (pulmonary function)24 and 48 weeks*

Change in Pulmonary Function as measured by percentage of Improving or worsening FVC.

The FVC (Forced vital Capacity) is the total amount of air exhaled during the FEV (Forced expiratory volume) test.

% Improving/worsening FVC. Analyzed as an ordinal outcome.

\*PFT (pulmonary function test) results will only be analyzed as available under standard of care.

Combined Response Index in diffuse cutaneous systemic sclerosis score (CRISS)Baseline (week 0), and at 24, 48 weeks

To define disease progression

CRISS score: It is calculated according to changes from the start of a study, compared to an endpoint, by using the modified Rodnan Skin Score (mRSS), the Health Assessment Questionnaire - Disability Index (HAQ-DI), patient and physician global assessment of scleroderma-related health, and forced vital capacity. A CRISS score of ≥ 0.6 indicates likelihood that a patient improved on treatment.

Change in skin thickness over time measured by modified Rodnan Skin Score12 weeks and 36 weeks

modified Rodnan Skin Score (mRSS): is a standard outcome measure for skin disease in SSc and calculated by measuring skin thickness in 17 different body sites (each site scored 0-3, with a total possible additive score of 51). A higher skin score (or a higher "skin thickness") and progression of this score, is predictive of internal organ involvement and mortality. While a lower or improving (lessening) score is associated with favorable outcomes, including better survival.

Change in the diffusing capacity for carbon monoxide (pulmonary function)24 and 48 weeks*

Change in Pulmonary Function as measured by percentage of Improving or worsening DLCO.

The DLCO (diffusing capacity of the lungs for carbon monoxide) measures the ability of the lungs to transfer gas from inhaled air to the red blood cells in pulmonary capillaries.

% Improving/worsening DLCO. Analyzed as an ordinal outcome.

\*PFT (pulmonary function test) results will only be analyzed as available under standard of care.

Change in patient global assessment of health status12, 24, 36, and 48 weeks

Measured on a Visual Analogue Scale (VAS) from 0-100, with 0 being no overall effect of disease on participant, and 100 being the worst possible overall effect of disease on participant.

This is a patient reported outcome.

Change in physician global assessment of disease severity12, 24, 36, and 48 weeks

Measured on a Visual Analogue Scale (VAS) from 0-10, with 0 being no disease severity and 10 being the worst possible disease severity.

Change in physician global assessment of disease damage12, 24, 36, and 48 weeks

Measured on a Visual Analogue Scale (VAS) from 0-10, with 0 being no disease damage and 10 being the worst possible disease damage.

Change in serum concentrations of Erythrocyte Sedimentation Rate12, 24, 36, and 48 weeks

Change in serum concentrations of the acute phase reactant, ESR

Reference ranges:

Male: 0-10 mm/h Female: 0-20 mm/h

A faster-than-normal rate may indicate inflammation in the body. Inflammation is part of the immune response system. The higher the number, the higher the likelihood of inflammation.

Change in physician global assessment of disease activity12, 24, 36, and 48 weeks.

Measured on a Visual Analogue Scale (VAS) from 0-10, with 0 being no disease activity and 10 being the worst possible disease activity.

Change in serum concentrations C-Reactive Protein12, 24, 36, and 48 weeks

Change in serum concentrations of the acute phase reactant, CRP

CRP aids in the evaluation of stress, trauma, infection, inflammation, surgery \& associated diseases. Blood levels of C-Reactive Protein (CRP) are known to rise in acute disease to a level of up to 50 mg/L in the presence of slight to moderate inflammatory process. Values \>50 mg/L indicate high and extensive inflammatory activity.

Trial Locations

Locations (1)

Rheumatology Clinic, St. Joseph's Health Care

🇨🇦

London, Ontario, Canada

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