RECRUITING

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

Study Overview

This clinical trial focuses on testing the efficacy of different digital interventions to promote re-engagement in cancer-related long-term follow-up care for adolescent and young adult (AYA) survivors of childhood cancer.

Description

Background: Kohlmeier-Degos (KD) is a rare disease that causes inflammation and blood clots, leading to blockages in small blood vessels. These blockages can result in K-D lesions throughout the body, affecting the skin, lungs, heart, spinal cord, and brain. KD can be fatal. No treatment exists for this disease. Objective: To test a study drug (baricitinib) in people with brain and spine lesions caused by KD disease. Baricitinib is FDA approved to treat other disorders but has not yet been tried in people with KD. Eligibility: People aged 18 years or older with KD-related lesions in the brain and spine. Design: Participants will be screened; they will have a physical exam with blood tests. They will also have a baseline visit that may include multiple tests, such as imaging scans of the brain and spine; a lumbar puncture to collect fluid from the spinal canal; and a meeting with a neurologist. They will fill out a questionnaire about their health. They will continue to take their normal medications throughout the study. Baricitinib is a tablet taken by mouth. Participants will remain on their normal medications for 12 weeks after their baseline visit. Then they will also take the study drug once a day at home for 24 weeks. Participants will have clinic visits every few weeks for up to 40 weeks. Some visits may take 1 to 4 days. Baseline tests will be repeated 3 more times during study visits. Other visits will require only blood tests; these may be done by local labs that will send the samples to NIH; 2 visits may be done via telehealth....

Official Title

A Phase IIa Trial of Baricitinib in the Treatment of Kohlmeier-Degos Disease Patients With Neurological Involvement

Quick Facts

Study Start:2025-04-16
Study Completion:2030-12-15
Study Type:Not specified
Phase:Not Applicable
Enrollment:Not specified
Status:RECRUITING

Study ID

NCT06923072

Participation Criteria

Researchers look for people who fit a certain description, called eligibility criteria. Some examples of these criteria are a person's general health condition or prior treatments.

Ages Eligible for Study:18 Years to 99 Years
Sexes Eligible for Study:ALL
Accepts Healthy Volunteers:No
Standard Ages:ADULT, OLDER_ADULT
Inclusion CriteriaExclusion Criteria
  1. * Provision of signed and dated informed consent form by the subject or Legally Authorized Representative (LAR).
  2. * Stated willingness to comply with all study procedures and availability for the duration of the study.
  3. * Male or female, aged 18 or older.
  4. * Subjects diagnosed with systemic Degos disease, who manifest neurologic abnormalities observed clinically, radiologically or in abnormal laboratory findings.
  5. * Ability to take oral medication and be willing to adhere to the baricitinib regimen.
  6. * 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.
  7. * Ability of subject or LAR to understand and the willingness to sign a written informed consent document.
  1. * Active infection not responding to appropriate therapy
  2. * Hemoglobin \<7 g/dL
  3. * Platelet counts \< 50 K /mcL
  4. * Neutropenia (ANC \<0.5 x k/mcL)
  5. * Lymphopenia (Absolute Lymphocyte Count \[ALC\] \<0.2x k/mcL)
  6. * Liver function tests (LFTs \> 2x time upper limit of normal)
  7. * Estimated Glomerular Filtration Rate (eGFR)/Creatinine (Cr \< 30 mL/min)
  8. * 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.
  9. * Have had symptomatic herpes zoster infection within 12 weeks prior to a enrolling in the study .
  10. * Have had household contact with a person with active TB and did not receive appropriate and documented prophylaxis for TB.
  11. * Have evidence of active TB or latent TB
  12. * 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
  13. * 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 .
  14. * Breast feeding
  15. * Pregnancy

Contacts and Locations

Study Contact

Jayson J Grey, R.N.
CONTACT
(301) 318-0338
jayson.grey@nih.gov
Cornelia D Cudrici, M.D.
CONTACT
(301) 451-7070
cornelia.cudrici@nih.gov

Principal Investigator

Cornelia D Cudrici, M.D.
PRINCIPAL_INVESTIGATOR
National Heart, Lung, and Blood Institute (NHLBI)

Study Locations (Sites)

National Institutes of Health Clinical Center
Bethesda, Maryland, 20892
United States

Collaborators and Investigators

Sponsor: National Heart, Lung, and Blood Institute (NHLBI)

  • Cornelia D Cudrici, M.D., PRINCIPAL_INVESTIGATOR, National Heart, Lung, and Blood Institute (NHLBI)

Study Record Dates

These dates track the progress of study record and summary results submissions to ClinicalTrials.gov. Study records and reported results are reviewed by the National Library of Medicine (NLM) to make sure they meet specific quality control standards before being posted on the public website.

Study Registration Dates

Study Start Date2025-04-16
Study Completion Date2030-12-15

Study Record Updates

Study Start Date2025-04-16
Study Completion Date2030-12-15

Terms related to this study

Keywords Provided by Researchers

  • Degos Disease
  • Kohlmeier-Degos
  • Baricitinib
  • Neurological involvement of Degos Disease

Additional Relevant MeSH Terms

  • Kohlmeier-Degos Disease
  • Malignant Atrophic Papulosis
  • Degos Disease
  • Papulosis, Malignant Atrophic