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Evaluating the Safety and Tolerability of Baricitinib in Patients With Job Syndrome With Lupus-Like Disease and/or Atopic Dermatitis | Clinical Trials | Clareo Health

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Evaluating the Safety and Tolerability of Baricitinib in Patients With Job Syndrome With Lupus-Like Disease and/or Atopic Dermatitis

A Pilot Study to Evaluate the Safety and Tolerability of Baricitinib in Patients With Job s Syndrome With Lupus-like Disease and/or Atopic Dermatitis

NCT07262983•National Institute of Allergy and Infectious Diseases (NIAID)

View on ClinicalTrials.gov

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....

Detailed Description

Study Description: This is an open-label, dose-titration, pilot trial to assess the safety of the Janus-associated kinase (JAK) inhibitor baricitinib in individuals with Job s syndrome with lupus-like features and/or atopic dermatitis (AD). Eligible participants will complete screening 3 months prior to the start of the trial and keep track of their infections and eczema to monitor Job s disease activity and ensure disease stability prior to initiation of baricitinib treatment. Those with stable disease will proceed to open-label treatment with baricitinib for 180 days. Primary Objective: To determine the safety and tolerability of JAK inhibitor treatment (baricitinib) in patients with Job s syndrome with lupus-like disease and/or AD. Secondary Objectives: 1. To assess changes in Candida and/or bacterial infections. 2. To evaluate the effect of baricitinib on lupus-like symptoms. 3. To evaluate the effect of baricitinib on AD. 4. To investigate the effect of baricitinib on quality of life (QOL). Exploratory Objectives: To investigate the effect of baricitinib on improving histologic and immunologic abnormalities in blood and affected organs and tissues. Primary Endpoints: 1\. Incidence of serious adverse events (SAEs), adverse events (AEs) requiring study drug discontinuation. Secondary Endpoints: 1. Incidence of Candida and/or bacterial infections. 2. Mean change in Cutaneous Lupus Erythematosus Disease Area and Severity Index (CLASI) score from day -90 to day 0 and then from day 0 to days 90 and 180. 3. Mean change in Eczema Area and Severity Index (EASI) score from day -90 to day 0 and then from day 0 to days 90 and 180. 4. Mean change in Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) score from day -90 to day 0 and then from day 0 to days 90 and 180. 5. Mean change in the Physician Global Assessment (PGA) score from day -90 to day 0 and then from day 0 to days 90 and 180. 6. Mean change in the 36-Item Short Form Survey (SF-36) score (adults) from day -90 to day 0 and then from day 0 to days 90 and 180. 7. Mean change in the Patient Reported Outcomes Measurement Information System (PROMIS) score from day -90 to day 0 and then from day 0 to days 90 and 180. 8. Mean change in the Dermatology Life Quality Index (DLQI) score (adults) from day -90 to day 0 and then from day 0 to days 90 and 180. 9. Mean change in the Children s Dermatology Life Quality Index (CDLQI) score (pediatrics) from day -90 to day 0 and then from day 0 to days 90 and 180. 10. Mean change in the Pediatric Quality of Life Inventory (PedsQL) score (pediatrics) from day -90 to day 0 and then from day 0 to days 90 and 180. Exploratory Endpoints: 1. Changes in serum cytokines and chemokines between day -90 to day 0 and then day 0 to days 90 and 180. 2. Changes in neutrophil extracellular trap (NET) complexes between days -90 to day 0 and then from day 0 to days 90, and 180. 3. Changes in Transcriptional alterations and pathway analysis using scRNAseq on peripheral blood mononuclear cells (PBMCs) and neutrophils between days -90 to day 0 and then from day 0 to days 90, and 180. 4. Changes in skin biopsy - histopathology and anatomic pathology from day 0 to day 180. 5. Changes in immunophenotyping between days -90 to day 0 and then from day 0 to days 90, and 180. 6. Changes in phospho-signal transducer and activator of transcription (STAT) flow cytometry between days -90 to day 0 and then from day 0 to days 90, and 180. 7. Changes in arterial stiffness- assessment of vascular functions (assessed via cardio-ankle vascular index \[CAVI\]) between day -90 and day 0, and then between day 0 and days 90 and 180. 8. Changes in autoantibody levels from day -90 to day 0 and then from day 0 to days 90 and 180. 9. Changes in skin microbiome over the 180-day treatment period. 10. Changes in serum metabolomics from day -90 to day 0 and then from day 0 to days 90 and 180. 11. Changes in serum proteomics from day -90 to day 0 and then from day 0 to days 90 and 180.

Eligibility

Age

12 - 120 years

Sex

ALL

Healthy Volunteers

Inclusion Criteria

•To be eligible to participate in this study, an individual must meet all of the following criteria:
•1. Must be able to understand and provide informed consent or assent.
•2. Aged \>=12 years.
•3. Documented STAT3 variant causing hyper-IgE syndrome.
•4. Enrollment in NIH protocol 00-I-0159, Natural History, Management, and Genetics of the Hyperimmunoglobulin E Recurrent Infection Syndrome (HIES).
•a. Presence of SLE and/or AD as follows: SLE patients should meet either Systemic Lupus International Collaborating Clinics (SLICC) or 2019 American College of Rheumatology (ACR)/European League Against Rheumatism (EULAR) SLE classification criteria. AD is defined as EASI tool score \>=16 and body surface area tool score of 10% at screening.
•5. Ability to take oral medication and be willing to adhere to the study intervention regimen.
•6. For individuals on glucocorticoids, the dose must be less than 10 mg daily and stable for the 30 days prior to Day 0.
•7. 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.
•8. 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.
+6 more criteria

Exclusion Criteria

•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.
•8. 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.
•9. History of alcohol or drug abuse within 6 months prior to Day 0.
+42 more criteria

Locations (1)

National Institutes of Health Clinical Center

Bethesda, Maryland, United States

Key Dates

Start Date

March 25, 2026

Primary Completion Date

May 1, 2030

Completion Date

October 1, 2030

Last Updated

March 20, 2026

Enrollment

ESTIMATED participants

Conditions

Hyper IgE Syndrome From STAT3 MutationJob s SyndromeHIESLupusAtopic Dermatitis

Interventions

baricitinib

DRUG

Contacts

Alexandra F Freeman, M.D.

CONTACT

(301) 594-9045 sarthak.gupta@nih.gov

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Data Source & Attribution

This clinical trial information is sourced from ClinicalTrials.gov, a service of the U.S. National Institutes of Health.

ClinicalTrials.gov last update: March 20, 2026Data synced to Clareo: March 29, 2026

Modifications: This data has been reformatted for display purposes. Eligibility criteria have been parsed into inclusion/exclusion sections. Location data has been geocoded to enable distance-based search. For the authoritative and most current information, please visit ClinicalTrials.gov.

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Evaluating the Safety and Tolerability of Baricitinib in Patients With Job Syndrome With Lupus-Like Disease and/or Atopic Dermatitis

Inclusion Criteria

Exclusion Criteria

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