RecruitingPhase 2

Rilonacept in Subjects With Cardiac Sarcoidosis

A RandomizEd PhAse II TrIal of Rilonacept in Subjects With Cardiac Sarcoidosis (REPAIR-CS)

Who can join

As published by the study on ClinicalTrials.gov. "Not stated" means the study hasn't said — ask the study team.

Age
18 Years to 80 Years
Sex
All sexes
Healthy volunteers
Not accepted
Compensation
Not stated

Other requirements are listed under Eligibility below. Only the study team can confirm whether you can join.

What to expect

No one can promise a trial will help you personally — that is what the research is trying to find out.

What participation involves

Not stated in the study description — ask the study team about visits and procedures.

Compensation
Not stated
  • Study-related exams and procedures are typically provided at no cost. This is how many trials work in general — confirm the details for this study with the study team.
  • Participants are monitored closely by the research team throughout the study.
  • Some participants may receive access to an investigational treatment, which is still being studied.
  • Your participation helps researchers learn whether this approach could help future patients.

About this study

In plain language

This study tests whether adding rilonacept to standard treatment reduces heart muscle inflammation more than standard treatment alone after 24 weeks. It is for adults ages 18 to 80 with cardiac sarcoidosis, a disease that causes inflammation in the heart.

The primary objective of this study is to evaluate the effect of rilonacept, added to standard therapy and compared with standard therapy alone, on improvement in myocardial inflammation in subjects with cardiac sarcoidosis after 24 weeks of therapy.

Compensation is read from the study's own description and may be missing — always confirm with the study team.

Eligibility

Who can join

  • Be 18 to 80 years old.
  • Understand and sign the consent form and agree to follow study rules.
  • Have cardiac sarcoidosis diagnosed using the guidelines listed in the study.
  • Have a PET scan within 8 weeks before study group assignment showing active inflammation in at least 3 heart areas despite standard treatment.
  • Agree to wear a portable heart rhythm monitor at set times.
  • Women must be past menopause, meet the study’s permanent birth control rules, or be not pregnant or breastfeeding and use effective birth control if sexually active, from the first screening visit until 5 months after study drug treatment.
  • Men must have a documented vasectomy or use the required birth control with partners who could become pregnant. These rules and no sperm donation apply from study group assignment until 3 months after the last dose.
  • Routine adult vaccines must be up to date or offered at least 2 weeks before study group assignment, as judged by the study doctor.

Who can't join

  • Weigh more than 380 pounds; be pregnant or breastfeeding; refuse required birth control; or be unable or unwilling to give consent.
  • Have fear of enclosed spaces or trouble completing PET scans, or an allergy to rilonacept or its ingredients.
  • Have certain heart size findings, heart support devices, or other heart diseases that could explain the heart problems.
  • Have another illness needing immune-suppressing drugs, recent changes in these drugs, or use of certain immune treatments within restricted time periods. Planned TNF-blocking treatment is also excluded.
  • Have tuberculosis (TB) history, exposure, symptoms, or test results covered by the study’s rules; serious or repeated infections; high infection risk; active hepatitis B; or COVID-19 symptoms without a recent negative PCR test.
  • Have uncontrolled blood pressure, thyroid disease, or diabetes; severe kidney problems; or blood count or liver test results outside the study’s limits.
  • Have recent or planned major surgery, an organ transplant, or cancer within 5 years, with limited exceptions for certain cornea transplants and treated cancers.
  • Have recently used an experimental treatment, be in another treatment study, or have another condition the doctor believes could affect safety or study results.

Simplified for readability. The official criteria below are what the study team uses.

Official eligibility criteria

Inclusion Criteria: 1. Able to comprehend and willing to sign an ICF and to abide by the study restrictions and requirements 2. Age ≥ 18 years and ≤ 80 years 3. Female subjects must be: * postmenopausal, defined as at least 12 months post cessation of menses (without an alternative medical cause), or * permanently sterile following documented hysterectomy, bilateral salpingectomy, bilateral oophorectomy, or tubal ligation, or having a male partner with vasectomy as affirmed by the subject, or * nonpregnant, nonlactating, and having agreed to use an effective method of contraception (i.e., hormonal contraception, intrauterine device \[IUD\], or double barrier methods such as condom plus diaphragm or diaphragm plus spermicide or condom plus spermicide) from Screening Visit 1 until 5 months after study drug administration, if sexually active. 4. Male subjects must have documented vasectomy or must use double barrier methods of contraception (such as condom plus diaphragm or diaphragm plus spermicide or condom plus spermicide) or use condoms plus hormonal contraceptives or condoms plus IUD with their female partners of childbearing potential from randomization to 3 months after the last dose of study drug administration. Male subjects must agree to refrain from donating sperm during this time period. 5. Routine adult vaccinations should be up to date and/or offered at least 2 weeks prior to randomization according to regional and national guidelines based on medical history or presence of risk factors, in the opinion of the Investigator. Cardiac Inclusion Criteria: 6. Has a diagnosis of cardiac sarcoidosis by the Heart Rhythm Society (HRS) expert consensus statement on the diagnosis and management of arrhythmias associated with cardiac sarcoidosis, or the Japanese Circulation Society 2016 Guideline on diagnosis and treatment of cardiac sarcoidosis (Terasaki 2019) 7. Three or more segments of active FDG uptake on PET scan within 8 weeks of randomization, despite standard therapy 8. Willing to wear an ambulatory cardiac rhythm monitor at the specified timepoints Exclusion Criteria 1. Unable or unwilling to provide informed consent 2. Weight \>380 pounds (172 kilograms) 3. Women who are pregnant or lactating or women of childbearing age who refuse to use a highly effective and medically acceptable form of contraception throughout the study 4. Planned to initiate TNF-α antagonist therapy over the course of the study. 5. Known claustrophobia, or difficulty completing prior PET scan procedure(s) 6. Left ventricular end-systolic diameter (LVESD) \> 60 mm on echocardiogram 7. Other systemic immune disorder(s) or other disorder(s) that require treatment with immunomodulators or immunosuppressants 8. Has received, or is scheduled to receive after randomization, mechanical circulatory support 9. Congenital, valvular, and/or coronary artery disease that could explain the severity of cardiac dysfunction 10. Known hypersensitivity to rilonacept (KPL-914) or to any of its excipients 11. Meets the following TB criteria: 1. History of active TB prior to screening OR 2. History of latent TB that was not adequately treated prior to screening OR 3. Signs or symptoms suggestive of active TB (e.g., new cough of \>14 days in duration or a change in chronic cough, persistent fever, unintentional weight loss, or night sweats) upon review of medical history and/or physical examination at screening OR 4. Recent close contact with a person with active TB OR 5. Positive or indeterminate Interferon Gamma Release Assay (IGRA) test results or results from another positive TB test at screening based on acceptable local clinical practice 12. Use of the following immunosuppressive or immunomodulatory therapies (see also Section 5.6 "Prior and Concomitant Therapy") within the timeframe prior to randomization as defined below for each drug class or category: 1. INCREASE in dose of existing immunosuppression/immunomodulation drug or INITIATION of new immunosuppression/immunomodulation drug in the one month prior to or ON or AFTER the date of the eligibility/baseline FDG-PET scan until the date of randomization. 2. Anakinra within 1 week prior to first dose of study drug; canakinumab within 8 weeks prior to the first dose of study drug; abatacept within 8 weeks prior to first dose of study drug; 3. TNF inhibitors within 2-8 weeks of or 5 half-lives (etanercept within 2 weeks; infliximab, certolizumab, golimumab, or adalimumab within 8 weeks) prior to first dose of study drug, whichever is longer. 4. Rituximab within 6 months prior to the first dose of study drug unless levels of CD20+ B cells have been assessed and have returned to normal. 5. Cyclosporine A (CsA) within 4 weeks prior to the first dose of study drug. 13. Received any investigational product within 30 days or 5 half-lives (if the half-life is known) of an investigational product (whichever is longer) prior to first dose of study drug 14. Concurrent enrollment in another clinical study, with the exception of observational studies 15. Uncontrolled hypertension (systolic blood pressure \>170 mmHg and diastolic blood pressure \>110 mmHg 16. Uncontrolled thyroid disease (serum TSH \< 0.1 mU/L or \> 10 mU/L) 17. Uncontrolled diabetes mellitus (serum glucose \> 180 mg/dL fasting or HbA1c \>9%) 18. Estimated glomerular filtration rate (eGFR) \<30mL/min 19. Major surgery within 8 weeks prior to screening or planned major surgery within 6 months after first dose of study drug. 20. Transplanted organs (except corneal transplant performed more than 3 months prior to first dose of study drug). 21. Severe active, recurrent, or chronic infection (per PI discretion), or any episode of infection requiring hospitalization or treatment with a course of IV antibiotics within 12 weeks before screening. Subjects with a history of severe opportunistic infection (per PI discretion) are also excluded from the study. 22. High risk of infection (e.g., history of hereditary or acquired immune deficiency disorder), a history of an infected joint prosthesis at any time with that prosthesis still in situ, leg ulcers, indwelling urinary catheter, or persistent or recurrent chest infections. 23. Chronic active HBV infection, defined as: 1. HBV surface antigen positive 2. HBV anti-core antibody positive but anti-surface antibody negative 24. Presence of symptoms indicative of COVID-19 infection (per PI discretion), unless a PCR test for COVID-19 has been reported as negative within the previous 7 days or is acquired prior to randomization. 25. History of cancer within the last 5 years from screening, except for basal and squamous cell carcinoma of the skin or in situ carcinoma of the cervix treated and considered cured. 26. Has screening laboratory test results meeting any of the following criteria: 1. Hemoglobin level \< 8.0 g/dL 2. WBC count \< 3.0 × 103/µL 3. Neutrophil count \<1.5 × 103/µL 4. Platelet count \< 100 × 103/µL 5. Total bilirubin level \>1.5 × ULN unless the test results are consistent with those for Gilbert's syndrome 6. AST or ALT values \> 2 × ULN 27. Any condition that, in the opinion of the investigator, could interfere with evaluation of the investigational product or interpretation of subject safety or confound the results of the study

Study sites (2)

MD

  • Johns Hopkins University · Baltimore, MD 21205Nisha Gilotra, M.D. · Study contactNisha Gilotra, M.D. · Principal investigatorMore trials in Baltimore

MN