a trial of baricitinib in patients with cardiac sarcoidosis

Conditions: Cardiac Sarcoidosis

Summary: the goal of this clinical trial is to learn if baricitinib in combination with a background steroid-sparing medication can treat active cardiac sarcoidosis in adults. the main question it aims to answer is: - in patients with active cardiac sarcoidosis, does treatment with baricitinib improve cardiac sarcoidosis disease activity as assessed by changes on cardiac fdg-pet/ct? participants will: * take baricitinib in combination with a steroid-sparing therapy for up to 16 weeks * visit the clinic every two to four weeks for checkups and tests * be asked to complete questionnaires to see how they feel on baricitinib and medication diaries to record when they take baricitinib


Eligibility criteria:
key inclusion criteria: * diagnosis of cardiac sarcoidosis based on one of the following pathways: * histological diagnosis * myocardial or extracardiac biopsy demonstrating non-caseating granuloma with no alternative cause identified and * abnormal fdg uptake on cardiac pet-ct conducted within six weeks of screening, in a pattern consistent with active cardiac sarcoidosis and * exclusion of other causes for cardiac manifestations * clinical diagnosis * one or more of the following is present: * steroid +/- immunosuppressant responsive cardiomyopathy or heart block * unexplained reduced lvef (\< 40%) and/or segmental wall motion abnormalities not related to coronary artery disease or another defined cause * unexplained sustained (spontaneous or induced) vt * mobitz type ii 2nd degree heart block or 3rd degree heart block * ct chest and/or fdg pet-ct showing features consistent with pulmonary sarcoidosis and/or hilar lymphadenopathy and * abnormal fdg uptake on cardiac pet-ct conducted within 6 weeks of screening, in a pattern consistent with active cardiac sarcoidosis and * exclusion of other causes for cardiac manifestations * active cardiac sarcoidosis based on abnormal fdg uptake on cardiac pet-ct conducted within six weeks of screening, in a pattern consistent with active cardiac sarcoidosis * no current treatment with immunosuppressive medications other than a steroid-sparing medication (including methotrexate, leflunomide, azathioprine, or mycophenolate mofetil), and/or prednisone (or equivalent) at a dose of ≤ 20mg daily at baseline key exclusion criteria: * receipt of a non-biologic dmard or immunosuppressive agent other than methotrexate, leflunomide, azathioprine, mycophenolate mofetil, hydroxychloroquine, or glucocorticoids within 28 days prior to screening * receipt of a bdmard or tsdmard, including non-depleting b-cell-directed therapy (eg, belimumab), t cell costimulatory blockade (eg, abatacept), tnf-alpha inhibition (eg, infliximab, adalimumab, etanercept, golimumab, certolizumab pegol), interleukin-6 inhibition (eg, tocilizumab, sarilumab), interleukin-1 inhibition (eg, anakinra), jak inhibition (eg, tofacitinib, upadacitinib, baricitinib), or other biologic immunomodulatory agent within 28 days prior to screening * receipt of any biologic b cell-depleting therapy (eg, rituximab, ocrelizumab, obinutuzumab, ofatumumab, inebilizumab) in the 6 months prior to screening; receipt of such a b cell-depleting agent in the period 6-12 months prior to screening is exclusionary unless b cell counts have returned to ≥ lln * history of venous thromboembolism (vte) or an increased risk for vte * current smoking * estimated glomerular filtration rate \< 30 ml/min/1.73 m2 by modification of diet in renal disease study (mdrd) equation * blood tests at screening that meet any of the following criteria: * hemoglobin \< 7.5 g/dl * neutrophils \< 1000/mm3 * absolute lymphocyte count \< 500/mm3 * platelets \< 100 x 109/l * subjects with the following abnormal liver function tests: * aspartate aminotransferase (ast) \> 2x uln * alanine aminotransferase (alt) \> 2x uln * total bilirubin (tbl) \> 2x uln unless ast, alt, and hemoglobin are within central laboratory normal range and the patient has a known history of gilbert syndrome * active, clinically significant infection at the time of screening * active malignancy or history of malignancy that was active within the last 5 years, except as follows: * in situ carcinoma of the cervix following apparently curative therapy \> 12 months prior to screening, * cutaneous basal cell or squamous cell carcinoma following apparently curative therapy, or * prostate cancer treated with radical prostatectomy or radiation therapy with curative intent \> 3 years prior to screening and without known recurrence or current treatment

Keywords: baricitinib sarcoidosis cardiac sarcoidosis janus kinase inhibitor jak inhibitor