Evaluating the Safety and Tolerability of Baricitinib in Patients With Job Syndrome With Lupus-Like Disease and/or Atopic Dermatitis
Eligible age
12–120 yrs
Accepts
All genders
Locations
1 state
Healthy volunteers
No
See if you qualify for this study
Answer a few quick questions about your location and health. Takes about a minute.
About this study
Background: Autosomal dominant hyper-IgE syndrome (HIES), also called Job syndrome, is a genetic disorder that affects the immune system. It can cause skin and lung infections and problems with blood vessels, connective tissues, and bones. People with HIES often have lupus-like disease or atopic dermatitis (skin rash). Researchers want to know if a drug approved to treat other immune system diseases (baricitinib) can help people with HIES. Objective: To test baricitinib in people with HIES with lupus-like disease or skin rash. Eligibility: People aged 12 years and older with HIES with lupus-like disease or skin rash. Design: Participants will have 5 clinic visits, 4 remote visits, and 2 phone visits in 9 months. Participants will be screened. They will have a physical exam with blood and urine tests. They will have tests of the speed and pressure of blood flow through their body: Blood pressure cuffs will be placed on each arm and leg; electrodes will be placed on the wrists and a microphone on the chest. The study has a 3-month lead-in period. Participants will not take the study drug during this time. They will continue with their usual medical care. They will have 2 phone calls with the study team. Baricitinib is a tablet taken by mouth. Participants will take 1 or 2 tablets by mouth every day for 6 months. They will start with a low dose and may increase to a higher dose. Blood and urine tests will be repeated during each study visit. Other tests may also be repeated during some visits. A skin sample may also be taken....
Sponsor: National Institute of Allergy and Infectious Diseases (NIAID)
You may not qualify if…
- ✕ An individual who meets any of the following criteria will be excluded from participation in this study:
- ✕ 1. Known history of hypersensitivity to baricitinib or other JAK inhibitors.
- ✕ 2. Current or recent use of any investigational drug/intervention (within 6 months or 5 half-lives, whichever is longer, prior to Day 0) except for COVID-19 vaccines or therapies that have been granted an FDA emergency authorization.
- ✕ 3. Scheduled to participate in another clinical study involving an investigational drug during the course of this study.
- ✕ 4. Use of systemic immunosuppressive or immune-modulating agents within 90 days prior to Day 0, except systemic steroids \<=10 mg of prednisone equivalent per day.
- ✕ 5. Current or prior treatment with rituximab in the 6 months prior to Day 0.
- ✕ 6. Current treatment with methotrexate, mycophenolate mofetil, other less common immunomodulatory drugs such as those falling into the class of disease-modifying antirheumatic drugs (DMARDs), belimumab, and other immunosuppressive biologics not otherwise specified herein. Participants previously on methotrexate, mycophenolate mofetil, azathioprine, tacrolimus, cyclosporine, or belimumab, other immunosuppressive biologics, or DMARDs should have been withdrawn from the drug for at least 90 days prior to Day 0.
- ✕ 7. Treatment with cyclophosphamide and pulse methylprednisolone within 6 months prior to Day 0.
Where it's recruiting
Bethesda
Source: ClinicalTrials.gov · NCT07262983 · last updated 2026-06-24