Rituximab for the Treatment of Refractory Cardiac Sarcoidosis: A Single-Center Experience

MOHAMED ELWAZIR, MEGAN L. KRAUSE, JOHN P. BOIS, GEORGIOS CHRISTOPOULOS, AYSE T. KENDI, JR LESLIE T. COOPER, HAYAN JOUNI, OMAR F. ABOUEZZEDDINE, PANITHAYA CHAREONTHAITAWEE, MOHAMED ABDELSHAFEE, SHREYASEE AMIN

Research output: Contribution to journalArticlepeer-review

Abstract

Background: We sought to examine the effect of anti–B-cell therapy (rituximab) on cardiac inflammation and function in corticosteroid-refractory cardiac sarcoidosis. Cardiac sarcoidosis (CS) is a rare cause of cardiomyopathy characterized by granulomatous inflammation involving the myocardium. Although typically responsive to corticosteroid treatment, there is a critical need for identifying effective steroid-sparing agents for disease control. Despite increasing evidence on the role of B cells in the pathogenesis of sarcoidosis, there is limited data on the efficacy of anti–B-cell therapy, specifically rituximab, for controlling CS. Methods and Results: We reviewed the clinical experience at a tertiary care referral center of all patients with CS who received rituximab after failing to improve with initial immunosuppression therapy, which included corticosteroids. Fluorodeoxyglucose positron emission tomography (FDG PET/CT) images before and after rituximab treatment were evaluated. All images were interpreted by 2 experienced nuclear medicine trained physicians. We identified 7 patients (5 men, 2 women; mean age at diagnosis, 49.0 ± 7.9 years) with active CS who were treated with rituximab. The median length of follow-up was 5.1 years. All individuals, but 1, had received prior steroid-sparing agents in addition to corticosteroids. Rituximab was administered either as 1000 mg intravenously ×1 or ×2 doses, separated by 2 weeks. Repeat dosing, if appropriate, was considered after 6 months. All tolerated the infusions well. Inflammation as assessed by maximum standardized uptake value on cardiac FDG PET/CT uptake significantly decreased in 6 of 7 patients (median 6.0–4.5, Wilcoxon signed rank z –1.8593, W 3), whereas the left ventricular ejection fraction improved or stabilized in 4 patients but decreased in 3. The mean left ventricular ejection fraction was 40.1% and 43.3% before and after treatment, respectively (P = .28). Three patients reported improved physical capacity, and 5 patients showed improved arrhythmic burden on Holter monitoring or implantable cardioverter-defibrillator interrogation. One patient subsequently developed a fungal catheter-associated infection and sepsis requiring discontinuation. Conclusions: Rituximab was well-tolerated and seemed to decrease inflammation, as assessed by cardiac FDG PET/CT in all but 1 patient with active CS. These data suggest that rituximab may be a promising therapeutic option for CS, which deserves merits further study.

Original languageEnglish (US)
JournalJournal of Cardiac Failure
DOIs
StateAccepted/In press - 2021

Keywords

  • Cardiac sarcoidosis
  • positron emission tomography
  • rituximab

ASJC Scopus subject areas

  • Cardiology and Cardiovascular Medicine

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