Baricitinib in the Treatment of Kohlmeier-Degos Disease in Patients With Neurological Involvement

Launched by NATIONAL HEART, LUNG, AND BLOOD INSTITUTE (NHLBI) · Apr 10, 2025

Keywords

ClinConnect Summary

This clinical trial is studying a medication called baricitinib for treating Kohlmeier-Degos (KD) disease, which causes serious issues like inflammation and blood clots that can affect various parts of the body, including the brain and spine. Currently, there are no available treatments for KD, so this study aims to see if baricitinib, which is already approved for other conditions, can help patients with brain and spine lesions caused by this rare disease.

To be eligible for the trial, participants must be 18 years or older and have been diagnosed with KD-related neurological problems. They will undergo several tests, including physical exams and imaging scans, to assess their condition. If they join the study, participants will take baricitinib daily for 24 weeks while continuing their regular medications. They will have regular clinic visits every few weeks for up to 40 weeks to monitor their health. This trial is currently recruiting participants, and it’s important to note that those with certain health issues or conditions may not be able to participate.

Gender

ALL

Eligibility criteria

• * INCLUSION CRITERIA:
• In order to be eligible to participate in this study, an individual must meet all of the following criteria:
• • Provision of signed and dated informed consent form by the subject or Legally Authorized Representative (LAR).
• • Stated willingness to comply with all study procedures and availability for the duration of the study.
• • Male or female, aged 18 or older.
• • Subjects diagnosed with systemic Degos disease, who manifest neurologic abnormalities observed clinically, radiologically or in abnormal laboratory findings.
• • Ability to take oral medication and be willing to adhere to the baricitinib regimen.
• • For female patients of reproductive potential, non-pregnant, non-breastfeeding: agree to use of highly effective contraception for the duration of the study and 30 days after the last dose.
• • Ability of subject or LAR to understand and the willingness to sign a written informed consent document.
• EXCLUSION CRITERIA:
• • Active infection not responding to appropriate therapy
• • Hemoglobin \<7 g/dL
• • Platelet counts \< 50 K /mcL
• • Neutropenia (ANC \<0.5 x k/mcL)
• • Lymphopenia (Absolute Lymphocyte Count \[ALC\] \<0.2x k/mcL)
• • Liver function tests (LFTs \> 2x time upper limit of normal)
• • Estimated Glomerular Filtration Rate (eGFR)/Creatinine (Cr \< 30 mL/min)
• • Have experienced any of the following within 12 weeks of screening: VTE (DVT/pulmonary embolism \[PE\]), myocardial infarction (MI), unstable ischemic heart disease, stroke, or New York Heart Association Stage III/IV heart failure.
• • Have had symptomatic herpes zoster infection within 12 weeks prior to a enrolling in the study .
• • Have had household contact with a person with active TB and did not receive appropriate and documented prophylaxis for TB.
• • Have evidence of active TB or latent TB
• • Have been exposed to a live vaccine within 12 weeks of baricitinib treatment or are expected to need/receive a live vaccine during the course of the study
• • No gadolinium based contrast agent exposure is permitted if eGFR \< 30 mL/min/1.73m\^2 using the CKD-EPI equation measured within 5 days .
• • Breast feeding
• • Pregnancy