Hyper IgE Syndrome From STAT3 Mutation study: Baricitinib
Official title Evaluating the Safety and Tolerability of Baricitinib in Patients With Job Syndrome With Lupus-Like Disease and/or Atopic Dermatitis
ClinicalTrials.gov ID: NCT07262983
What this study is testing
What is Baricitinib?
An investigational oral tablet being studied as a potential treatment for hyper‑IgE syndrome (Job syndrome) with lupus‑like disease or skin rash. Baricitinib is designed to inhibit Janus kinase (JAK) enzymes, which reduces signaling that drives inflammation.
Also referred to as Olumiant.
Plain-language explanation of the investigational treatment. It is being studied and is not an approved or proven therapy. The study team can confirm the details.
- What it's testing
- The study wants to see if baricitinib is safe and tolerable for people with Job syndrome who also have lupus‑like symptoms or atopic dermatitis.
- Who it's for
- People age 12 or older who have Job syndrome (hyper‑IgE syndrome) with lupus‑like disease or eczema.
- What's involved
- Participants will take baricitinib tablets daily for 6 months, starting with a low dose that may be increased. They will have regular clinic, remote, and phone visits for physical exams, blood and urine tests, and occasional skin samples.
- Time commitment
- The trial lasts about 9 months, including a 3‑month lead‑in period, with 5 in‑person clinic visits, 4 remote visits and 2 phone calls.
- Phase 1: an early, usually small safety study
A plain-language read of the study's public ClinicalTrials.gov listing. The study team confirms the details.
Who can take part
Ages 12 to 120
You may be able to join if
- Must be able to understand and provide informed consent or assent.
- Aged \>=12 years.
- Documented STAT3 variant causing hyper-IgE syndrome.
- Enrollment in NIH protocol 00-I-0159, Natural History, Management, and Genetics of the Hyperimmunoglobulin E Recurrent Infection Syndrome (HIES). a.
- Ability to take oral medication and be willing to adhere to the study intervention regimen.
You likely can't join if
- 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...
- 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...
- 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...
See the full eligibility criteria
Who can join
- Must be able to understand and provide informed consent or assent.
- Aged \>=12 years.
- Documented STAT3 variant causing hyper-IgE syndrome.
- Enrollment in NIH protocol 00-I-0159, Natural History, Management, and Genetics of the Hyperimmunoglobulin E Recurrent Infection Syndrome (HIES). a.
- 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.
- 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.
- Intrauterine device (IUD)
- Bilateral tubal ligation
- Abstinence
- Vasectomized partner
- Hormonal contraception used in combination with barrier method: progestogen containing (oral, intravaginal, transdermal) or progestogen-only (oral, injectable, implantable) starting 30 days prior to initiation of...
What rules you out
- 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...
- 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...
- 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.
- History of alcohol or drug abuse within 6 months prior to Day 0.
- Serum ALT \>=3 times ULN.
- Serum total bilirubin \>=2 times ULN.
- ANC \<=750 cells/microL.
- Hemoglobin \<=9.0 g/dL.
- Platelet count \<=100,000/microL.
- Serum creatinine \>=2 times ULN.
- Planned or anticipated major surgical procedure during the study.
- Plans to receive any live vaccines within 1 month of the anticipated first dose of baricitinib.
- Known or suspected immune-dysregulatory disorders besides Job s syndrome, lupus-like disease, and/or AD.
- Active invasive opportunistic infections (eg, non-TB mycobacterial infections, histoplasmosis, listeriosis, coccidioidomycosis, pneumocystis pneumonia, aspergillosis) despite infection resolution or otherwise recurrent...
- Known active TB. Participants with treated LTB will be eligible to participate. Participants with untreated LTB will not be excluded but will be evaluated by an infectious disease consultant and may become eligible for...
- Infection with HIV.
- Untreated infection with hepatitis B or C.
- Unwillingness to receive prophylactic entecavir (or similar), only for individuals with evidence of clearance of hepatitis B with positive hepatitis B core and surface antibody and negative hepatitis B surface antigen...
- BK or JC viremia at screening visit.
- Active infection that requires the use of oral or intravenous antimicrobials that remains unresolved at least 14 days prior to the administration of the first dose of study medication.
- Individuals with active renal or central nervous system disease or a high activity level in any organ system (except articular) that requires immediate immunosuppressive therapy as determined by the investigator.
- History of cancer, with the exceptions of basal cell carcinoma, localized squamous cell carcinoma of the skin, or in situ carcinoma of the cervix, provided the participant is in remission and curative therapy was...
- Planned or anticipated use of any prohibited medications and procedures during the study.
- Pregnancy or current breastfeeding.
- Currently receiving hemodialysis or peritoneal dialysis.
- Known coronary artery aneurysm.
- Known history of arterial or venous thrombosis or at high risk for clotting disorder.
- Known history of PE or DVT in the past.
- Psychiatric illness or history of medical non-compliance that the study team feels will make the individual unlikely to complete the study.
- Significant impairment of major organ function (lung, heart, liver, kidney) or any condition that, in the opinion of the investigator, would jeopardize the individual s safety following exposure to the study drug.
- Ischemic heart disease (eg, history of acute myocardial infarction).
- Heart failure.
- Cardiomyopathy.
- Severe valvular heart disease.
- Significant arrhythmias.
- Chronic renal failure.
- Cerebrovascular accident or transient ischemic attack.
- Uncontrolled diabetes mellitus.
- Uncontrolled hypertension.
- Current smokers or former smokers with less than 3 years since complete cessation and/or \>20 pack-years of smoking history.
- History of idiopathic GI perforation or diverticulitis with high risk of perforation.
- Treatment with strong organic anion transporter 3 inhibitors (OAT3) (eg, probenecid) due to drug interactions.
- Uncontrolled thyroid disease as per principal investigator or medically responsible investigator.
The study team makes the final eligibility decision.
Where it is enrolling
- Bethesda, Maryland, United States
Questions & answers
Do participants get paid in this trial?
This listing doesn't specify compensation. Many trials still reimburse travel or offer a stipend, so it's worth asking the study team when you connect.
Is it free to join, and do I need insurance?
Searching and applying through BridgeMD is free. In clinical trials the study-related treatment and visits are generally provided at no cost to you. You usually don't need insurance to take part, but confirm specifics with the study team.
How long does this study last?
The listing doesn't state an exact length. The study team walks you through the schedule and number of visits before you decide to enroll.
Who can join this trial?
This study is enrolling all sexes, 12 years to 120 years. The study team makes the final eligibility decision.
Where is this trial taking place?
This study is enrolling now at Bethesda, Maryland, United States. Enter your location above to see the nearest enrolling site and check your eligibility.
Explore other conditions
BridgeMD is an information and trial-matching tool that is independent of the study sponsor. Details come from ClinicalTrials.gov. The study team decides eligibility, and your own doctor gives medical advice.