Safety and Tolerability Study of Baricitinib in Patients Aged 12 and Older with Job Syndrome Featuring Lupus-Like Disease or Atopic Dermatitis

What this Trial Is About

Researchers are evaluating the safety and tolerability of baricitinib, a Janus-associated kinase JAK inhibitor, in people aged 12 and older with autosomal dominant hyper-IgE syndrome HIES, also called Job syndrome. This genetic disorder affects the immune system and can cause skin and lung infections, blood vessel, connective tissue, and bone problems, as well as lupus-like disease or atopic dermatitis skin rash. The study aims to better understand how baricitinib affects these conditions in this population. Participants will first undergo a 3-month lead-in period without taking the study drug, continuing their usual medical care while monitoring disease activity. After this, they will receive baricitinib orally once daily for 6 months, starting at a low dose that may be increased after safety evaluation. Throughout the study, they will have regular blood and urine tests, assessments of skin condition and lupus symptoms, and other evaluations including skin biopsies and vascular function tests. Participants will have a total of 5 clinic visits, 4 remote visits, and 2 phone calls over 9 months. During these visits, a range of tests will be performed, including physical exams, blood and urine tests, skin assessments, and monitoring of infections and disease activity. Researchers will measure safety outcomes such as serious adverse events and drug discontinuation due to side effects, as well as changes in infection rates, skin symptoms, lupus activity, quality of life, and various biomarkers. The study includes close monitoring to understand how baricitinib affects different aspects of the disease and patient well-being.

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