Diffuse Lung Disease: Sarcoidosis |

Multicenter Experience With Mycophenolate Mofetil in Cardiac Sarcoidosis FREE TO VIEW

Lynn Fussner, MD; Erin Karlstedt, MD; Nowell Fine, MD; Sanjay Kalra, MD; Eva Carmona, MD; James Utz, MD; Debra Isaac, MD; Leslie Cooper, MD
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Division of Pulmonary and Critical Care Medicine, Mayo Clinic, Rochester, MN

Copyright 2016, American College of Chest Physicians. All Rights Reserved.

Chest. 2016;150(4_S):512A. doi:10.1016/j.chest.2016.08.526
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SESSION TITLE: Sarcoidosis

SESSION TYPE: Original Investigation Slide

PRESENTED ON: Wednesday, October 26, 2016 at 07:30 AM - 08:30 AM

PURPOSE: Cardiac sarcoidosis (CS) is a potentially life-threatening cardiomyopathy, although limited data exist to guide pharmacotherapy. Corticosteroids presently constitute the mainstay of treatment, but are associated with significant toxicities. Mycophenolate mofetil (MMF) is a corticosteroid-sparing agent that may have a role in management of CS.

METHODS: Detailed retrospective chart review was performed to identify patients from two academic medical centers meeting 2014 Heart Rhythm Society Expert Consensus criteria for the diagnosis of CS, from 1994-2014. Patients receiving MMF for CS were included in this analysis. Data regarding demographics, disease characteristics, concurrent therapies, and composite endpoint of ventricular assist device (VAD), heart transplant, or death, were abstracted. Kaplan-Meier analyses were used to evaluate time-dependent variables.

RESULTS: A total of 33 patients were treated with MMF for a median of 22.0 months (IQR13.0-104.0). Median age at diagnosis was 51.0 years (IQR 47.0-58.0), 24 (73%) patients were male, and 26 (79%) were Caucasian. Cardiomyopathy with left ventricular ejection fraction (LVEF) <40% was the most common presenting manifestation, affecting 17 (52%) patients, followed by 12 (36%) with atrioventricular block, and 11 (33%) with ventricular arrhythmia; 6 (18%) had more than one of these at presentation. Only 3 (9%) patients had isolated cardiac involvement by sarcoidosis, while 28 (85%) also had pulmonary involvement. All but one patient (97%) initially received concurrent prednisone, 2 (6%) received concurrent anti-tumor necrosis factor-alpha therapy, and 1 (3%) received concurrent cyclosporine. Twenty (of 32, 63%) patients were tapered off prednisone, with median prednisone duration after MMF initiation of 15.0 months (IQR 7.1-33.5). Median time to worsening cardiomyopathy (clinical and/or 10% decrease in LVEF) was 28.0 months, while time to recurrent ventricular arrhythmia was 11.5 months. Six (18%) patients received a VAD, and 5 (15%) underwent transplantation (4 with preceding VAD). After MMF initiation, median time to the composite endpoint of VAD, heart transplant, or death, was 79.0 months. Three (9%) patients not receiving VAD or transplant changed therapy for apparent disease progression. Four (12%) patients stopped MMF due to adverse effects, including cytopenias (3), rash (1), and tremor (1).

CONCLUSIONS: In this multicenter analysis, MMF allowed a majority of patients with CS to be weaned off prednisone. Median survival without the composite endpoint of VAD, transplant, or death, was more than 6.5 years, suggesting a benefit of immunosuppression. Discontinuation of MMF for toxicity was uncommon.

CLINICAL IMPLICATIONS: Prospective studies are needed to better define the role of MMF in CS therapy.

DISCLOSURE: The following authors have nothing to disclose: Lynn Fussner, Erin Karlstedt, Nowell Fine, Sanjay Kalra, Eva Carmona, James Utz, Debra Isaac, Leslie Cooper

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