Actively Recruiting
Evaluating the Safety and Tolerability of Baricitinib in Patients With Job Syndrome With Lupus-Like Disease and/or Atopic Dermatitis
Led by National Institute of Allergy and Infectious Diseases (NIAID) · Updated on 2026-05-14
20
Participants Needed
1
Research Sites
228 weeks
Total Duration
On this page
AI-Summary
What this Trial Is About
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....
CONDITIONS
Official Title
Evaluating the Safety and Tolerability of Baricitinib in Patients With Job Syndrome With Lupus-Like Disease and/or Atopic Dermatitis
Who Can Participate
Eligibility Criteria
You may qualify if you...
- Able to understand and provide informed consent or assent
- Aged 12 years or older
- Documented STAT3 variant causing hyper-IgE syndrome
- Enrollment in NIH protocol 00-I-0159 for hyperimmunoglobulin E recurrent infection syndrome
- Diagnosed with systemic lupus erythematosus or atopic dermatitis (EASI score ≥16 and body surface area ≥10%) at screening
- Able to take oral medication and willing to follow study treatment
- If on glucocorticoids, dose less than 10 mg daily and stable for 30 days prior to study start
- If on hydroxychloroquine or other antimalarials, dose stable for 90 days prior to study start and within allowed maximum doses
- If on lipid-lowering medications, dose stable for 30 days prior to study start
- Individuals of reproductive potential must agree to use effective contraception during study
You will not qualify if you...
- Known allergy to baricitinib or other JAK inhibitors
- Use of investigational drugs within 6 months prior to study start (except authorized COVID-19 treatments)
- Participation in another clinical trial with investigational drugs during this study
- Use of systemic immunosuppressive agents within 90 days prior to study start, except low-dose steroids
- Treatment with rituximab within 6 months prior to study start
- Treatment with certain immunomodulatory or immunosuppressive drugs within 90 days prior to study start
- Treatment with cyclophosphamide or pulse methylprednisolone within 6 months prior to study start
- High cholesterol or triglyceride levels within 90 days prior to study start
- History of alcohol or drug abuse within 6 months prior to study start
- Significant laboratory abnormalities including elevated liver enzymes, low blood counts, or high creatinine
- Planned major surgery during the study
- Plans to receive live vaccines within 1 month before starting study drug
- Other immune disorders besides Job syndrome, lupus-like disease, or atopic dermatitis
- Active invasive or recurrent infections indicating immune compromise
- Active tuberculosis, HIV, or untreated hepatitis B or C infections
- Unwillingness to take prophylactic antiviral medication if indicated
- Active infection requiring antibiotics unresolved 14 days before study drug
- Active severe renal or central nervous system disease requiring immediate immunosuppressive therapy
- History of cancer except certain skin or cervical cancers in remission
- Use of prohibited medications during the study
- Pregnancy or breastfeeding
- Currently on dialysis
- Medical conditions or findings posing additional risks or interfering with study adherence, including coronary artery aneurysm, clotting disorders, prior blood clots, psychiatric illness affecting compliance, or major organ impairment
- Known increased risk factors for major adverse cardiovascular events, including ischemic heart disease, heart failure, cardiomyopathy, severe valve disease, arrhythmias, chronic renal failure, stroke, uncontrolled diabetes or hypertension, and smoking history
- History of gastrointestinal perforation or high-risk diverticulitis
- Use of strong organic anion transporter 3 inhibitors
- Uncontrolled thyroid disease as determined by study doctor
AI-Screening
AI-Powered Screening
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Trial Site Locations
Total: 1 location
1
National Institutes of Health Clinical Center
Bethesda, Maryland, United States, 20892
Actively Recruiting
Research Team
A
Alexandra F Freeman, M.D.
CONTACT
How is the study designed?
Study Type
INTERVENTIONAL
Masking
NONE
Allocation
NA
Model
SINGLE_GROUP
Primary Purpose
TREATMENT
Number of Arms
1
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