Actively Recruiting

Phase 2
Age: 4Years +
All Genders
ID04925375

Abatacept for Common Variable Immunodeficiency With Interstitial Lung Disease in Pediatric and Adult Patients

Led by Children's Hospital Medical Center, Cincinnati · Updated on 2025-11-06

38

Participants Needed

6

Research Sites

N/A

Total Duration

On this page

Sponsors

C

Children's Hospital Medical Center, Cincinnati

Lead Sponsor

B

Bristol-Myers Squibb

Collaborating Sponsor

AI-Summary

What this Trial Is About

Researchers are evaluating abatacept as a treatment for granulomatous-lymphocytic interstitial lung disease (GLILD) in patients with common variable immunodeficiency (CVID), a condition without a standard therapy. This multi-site, phase II randomized, blinded, placebo-controlled clinical trial includes both pediatric and adult participants to compare the effects of abatacept against placebo. The study aims to assess the efficacy of abatacept in this complex immune condition, funded by the FDA Office of Orphan Products Development. Participants are divided into two cohorts based on weight. Cohort 1 includes pediatric subjects weighing 50 kg or more and adults, randomized in a 1:2 ratio to receive either abatacept or placebo weekly for six months, followed by open-label abatacept for all. Cohort 2 includes pediatric subjects under 50 kg receiving open-label abatacept with dosing based on weight. After the initial 12 months, patients can opt to continue abatacept treatment for up to three years, with safety monitoring every three months. During the study, participants will undergo assessments including high-resolution chest CT scans, lung function tests such as forced vital capacity and forced expiratory volume, quality of life questionnaires, and monitoring for infections and adverse events at 6 and 12 months. Additional evaluations include pediatric growth measures and steroid use. Safety monitoring continues for those opting for extended treatment, ensuring a comprehensive overview of abatacept’s effects over time.

CONDITIONS

Brief Title

Abatacept for the Treatment of Common Variable Immunodeficiency With Interstitial Lung Disease

Who Can Participate

Age: 4Years +
All Genders

Eligibility Criteria

Eligible

You may qualify if you...

  • Diagnosis of common variable immunodeficiency (CVID) according to international consensus
  • Age 4 years or older
  • Serum IgG at least 2 standard deviations below age-adjusted normal
  • Decreased serum IgA and/or IgM
  • Abnormal specific antibody response to immunization
  • On replacement immunoglobulin therapy for at least 6 months and willing to maintain this during the study
  • Diagnosis of granulomatous-lymphocytic interstitial lung disease confirmed by lung biopsy
  • Persistence or worsening of interstitial lung disease shown on CT scans at least 6 months apart
  • Signed informed consent
  • Willingness to allow storage of biological specimens for future research
  • Female participants of childbearing potential must agree to effective birth control
  • Fertile, non-vasectomized males with female partners of childbearing potential must use condoms during the study and for 3 months after last dose
Not Eligible

You will not qualify if you...

  • History of hypersensitivity to abatacept or its components
  • Use of lymphocyte depleting agents within 6 months prior to study
  • Use of abatacept, cyclophosphamide, tumor necrosis factor inhibitors, or high-dose steroids in past 3 months
  • Recent start or increase of certain immune modulating drugs within 3 months prior to enrollment
  • History of HIV infection
  • Untreated chronic hepatitis B or C infection
  • Active tuberculosis or untreated latent TB
  • Persistent Epstein-Barr Virus load above threshold
  • Other uncontrolled infections
  • Live vaccine administered within 6 weeks before trial start
  • Malignancy within past year
  • Currently pregnant or breastfeeding
  • Life expectancy less than 1 month
  • Unwillingness or inability to self-administer or have caregiver administer subcutaneous injections
  • Other conditions deemed contraindicated by investigators

AI-Screening

AI-Powered Screening

Complete this quick 3-step screening to check your eligibility

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Your Study Journey

Screening

Duration - 2 to 4 weeks

Participants are screened for eligibility to participate in the trial.

1 screening and enrollment visit

Treatment

Duration - 12 months

Participants receive weekly subcutaneous injections of abatacept or placebo for the first 6 months. After 6 months, all participants receive abatacept weekly.

Weekly visits for up to 6 months, then weekly treatment visits up to 12 months

Follow-up

Duration - Up to 3 years

Participants who choose to continue abatacept after the initial 12 months are monitored for safety, including infections.

Safety monitoring visits approximately every 3 months

Trial Site Locations

Total: 6 locations

1

University of California, San Francisco

San Francisco, California, United States, 94143

Actively Recruiting

2

University of South Florida

Tampa, Florida, United States, 33620

Actively Recruiting

3

Lahey Hospital and Medical Center

Burlington, Massachusetts, United States, 01805

Actively Recruiting

4

Mayo Clinic

Rochester, Minnesota, United States, 55902

Actively Recruiting

5

Duke University Health System

Durham, North Carolina, United States, 27710

Actively Recruiting

6

Cincinnati Children's Hospital Medical Center

Cincinnati, Ohio, United States, 45229

Actively Recruiting

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Research Team

M

Michael Jordan

How is the study designed?

Study Type

INTERVENTIONAL

Masking

QUADRUPLE

Allocation

RANDOMIZED

Model

PARALLEL

Primary Purpose

TREATMENT

Number of Arms

2

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