Evaluating the Safety and Tolerability of Baricitinib in Patients With Job Syndrome With Lupus-Li… (NCT07262983) | Clinical Trial Compass
RecruitingPhase 1
Evaluating the Safety and Tolerability of Baricitinib in Patients With Job Syndrome With Lupus-Like Disease and/or Atopic Dermatitis
United States20 participantsStarted 2026-07-01
Plain-language summary
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....
Who can participate
Age range
12 Years – 120 Years
Sex
ALL
See this in plain English?
AI-rewrites the medical criteria so a patient or caregiver can understand them. Always confirm with the trial site.
Inclusion criteria
. Must be able to understand and provide informed consent or assent.
. Enrollment in NIH protocol 00-I-0159, Natural History, Management, and Genetics of the Hyperimmunoglobulin E Recurrent Infection Syndrome (HIES).
. Ability to take oral medication and be willing to adhere to the study intervention regimen.
. For individuals on glucocorticoids, the dose must be less than 10 mg daily and stable for the 30 days prior to Day 0.
. For individuals on hydroxychloroquine or other antimalarials such as chloroquine or quinacrine, the dose must have been stable for 90 days prior to Day 0. The maximum allowed dose is hydroxychloroquine 400 mg/day or 6.5 mg/kg/day, whichever is greater. The maximum allowed dose for chloroquine phosphate is 500 mg daily, and for quinacrine is 100 mg daily.
Questions worth asking your doctor
Bring these to your next appointment. They're a starting point for a shared conversation — not a sign you qualify or a recommendation to enrol.
1Since this is a Phase 1 trial primarily measuring serious adverse events and side effects serious enough to stop the drug, what does that mean for what's currently known — or not yet known — about whether baricitinib is safe for people with Job syndrome?
2Baricitinib is a JAK inhibitor already used for other conditions like rheumatoid arthritis — does my doctor think the existing safety data from those uses is reassuring enough given that this trial is still in early-phase testing for Job syndrome specifically?
3My situation involves both Job syndrome and either lupus-like disease or atopic dermatitis — does my doctor think my particular combination of symptoms makes this trial worth discussing as an option, or would standard treatments be a better starting point?
4Since the trial is still recruiting and in Phase 1, what would happen to my care if I enrolled and then had to stop the study drug due to a side effect — would I be able to go back to my current treatment plan?
5Given that Job syndrome caused by STAT3 mutations is a rare condition, how much experience does my doctor or this trial site have treating patients like me, and would that affect how closely I'd be monitored if I were to participate?
Generated to help you prepare — always confirm anything about your own eligibility and care with the study team and your doctor.
Questions for the trial coordinator
The trial coordinator is the person who runs the study day to day. These cover the practical side — logistics, costs, and what taking part would actually mean for your life. The study team confirms whether you meet the criteria; these are questions to ask, not a sign you qualify.
1What does taking part actually involve week to week — how many visits, where, and how long does each one take?
2What costs are covered by the study, and what might I have to pay for myself, including travel, parking, or time off work?
3What happens during screening, and what happens if the study team confirms I don't meet the criteria after those tests?
4Who pays for the scans, blood work, and other tests the trial requires — the study, my insurance, or me?
5How will being in the trial affect my regular care, and will my own doctor stay informed and involved?
6Can I leave the trial at any point if I change my mind, and what would happen to my care if I do?
A starting point for the conversation — always confirm anything about your own eligibility, costs, and care with the study team and your doctor.
What they're measuring
1
Incidence of SAEs, AEs requiring study drug discontinuation.
Timeframe: Through end of study
Trial details
NCT IDNCT07262983
SponsorNational Institute of Allergy and Infectious Diseases (NIAID)
. Individuals may be on lipid-lowering medications if initiated at least 90 days prior to Day 0, and the dose must be stable for 30 days prior to Day 0.
Exclusion criteria
. Known history of hypersensitivity to baricitinib or other JAK inhibitors.
. 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.
. Scheduled to participate in another clinical study involving an investigational drug during the course of this study.
. 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.
. Current or prior treatment with rituximab in the 6 months prior to Day 0.
. 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.
. Treatment with cyclophosphamide and pulse methylprednisolone within 6 months prior to Day 0.
. Hypercholesterolemia: Values after 8- to 12-hour fasting blood specimen: total cholesterol \>250 mg/dL or LDL \>180 mg/dL or hypertriglyceridemia (triglyceride \>300 mg/dL) within 90 days prior to Day 0.