Background/Purpose:   Cardiac sarcoidosis is a life threatening condition for which there is limited data to guide optimal steroid-sparing agents. B-cells have been reported to be involved in the pathogenesis of sarcoidosis and individual instances of successful rituximab use have been described.

Methods: We identified all patients at a single center with cardiac sarcoidosis (defined by endomyocardial biopsy or biopsy of different organ confirming sarcoidosis plus consistent cardiac imaging) treated with rituximab with at least 1 follow-up.

Results: Mean ± standard deviation age at diagnosis for the 3 men and 2 women identified was 50.9 ± 8.8 years. Median follow-up at our center for sarcoidosis was 2.7 years (range 1.3-7.1). One patient had isolated cardiac sarcoidosis. The remaining 4 had extracardiac involvement: thoracic lymphadenopathy (3, 60%), pulmonary (1, 20%), renal (1, 20%), parotid gland (1, 20%), and neurologic (1, 20%). Endomyocardial biopsies were positive in 2 of 3 patients. All had abnormal findings on PET and/or MRI. Four had cardiac involvement at the time of sarcoid diagnosis. Cardiac manifestations included both arrhythmia (high grade AV block, ventricular tachycardia) and heart failure. All 5 had an implantable cardioverter defibrillator (ICD) over their follow-up. All 5 received corticosteroids and had failed or were intolerant to at least 1 additional immunosuppressant before rituximab. Mycophenolate mofetil was the most common (4, 80%). Methotrexate was used in 2 (40%), azathioprine 1 (20%), infliximab 1 (20%), and leflunomide 1 (20%). Rituximab 1000 mg IV x 2 doses, 2 weeks apart, was given to 4 patients. One received only 1 dose due to insurance limitations. Three patients received 1000 mg IV x 2 doses after at least 6 month intervals, for a total of 2, 3 and 4 rounds, for each patient, respectively. The median follow-up following first rituximab was 0.8 (range 0.2-1.9) years. All received prednisone with doses successfully reduced over follow-up. Two also received methotrexate with rituximab, with one having to discontinue due to renal dysfunction. All had serial cardiac PETs. In all, there was qualitative improvement in inflammation as assessed by a decrease in FDG uptake. In one patient, all FDG uptake was eliminated in two serial cardiac PETs following the first and second round of rituximab. Due to insurance issues, the third round of rituximab was delayed to 10 months and with recurrence of FDG uptake that stabilized on subsequent cardiac PET. Three had improvement of ejection fraction as measured by cardiac PET following first rituximab infusion: 26% to 54%, 33% to 47%, and 32% to 40%. One had stability and 1 had worsening from 45% to 28% but the latter was performed in the setting of ectopic beats and echocardiogram performed at similar time points demonstrated stability. No individuals died during follow-up. One individual is currently listed as class IB for heart and kidney transplant. No side effects from rituximab were noted.

Conclusion:   Rituximab for treatment refractory/steroid-intolerant cardiac sarcoidosis appears to be effective and well-tolerated. Further work is needed to determine its ideal position in a regimen to treat cardiac sarcoidosis.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/rituximab-in-refractory-cardiac-sarcoidosis-single-center-experience/