Long‐Term Corticosteroid‐Sparing Immunosuppression for Cardiac Sarcoidosis

Background Long‐term corticosteroid therapy is the standard of care for treatment of cardiac sarcoidosis (CS). The efficacy of long‐term corticosteroid‐sparing immunosuppression in CS is unknown. The goal of this study was to assess the efficacy of methotrexate with or without adalimumab for long‐te...

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Main Authors: David G. Rosenthal, Purvi Parwani, Tyler O. Murray, Bradley J. Petek, Bryan S. Benn, Teresa De Marco, Edward P. Gerstenfeld, Munir Janmohamed, Liviu Klein, Byron K. Lee, Joshua D. Moss, Melvin M. Scheinman, Henry H. Hsia, Van Selby, Laura L. Koth, Miguel H. Pampaloni, Julie Zikherman, Vasanth Vedantham
Format: Article
Language:English
Published: Wiley 2019-09-01
Series:Journal of the American Heart Association: Cardiovascular and Cerebrovascular Disease
Subjects:
Online Access:https://www.ahajournals.org/doi/10.1161/JAHA.118.010952
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author David G. Rosenthal
Purvi Parwani
Tyler O. Murray
Bradley J. Petek
Bryan S. Benn
Teresa De Marco
Edward P. Gerstenfeld
Munir Janmohamed
Liviu Klein
Byron K. Lee
Joshua D. Moss
Melvin M. Scheinman
Henry H. Hsia
Van Selby
Laura L. Koth
Miguel H. Pampaloni
Julie Zikherman
Vasanth Vedantham
author_facet David G. Rosenthal
Purvi Parwani
Tyler O. Murray
Bradley J. Petek
Bryan S. Benn
Teresa De Marco
Edward P. Gerstenfeld
Munir Janmohamed
Liviu Klein
Byron K. Lee
Joshua D. Moss
Melvin M. Scheinman
Henry H. Hsia
Van Selby
Laura L. Koth
Miguel H. Pampaloni
Julie Zikherman
Vasanth Vedantham
author_sort David G. Rosenthal
collection DOAJ
description Background Long‐term corticosteroid therapy is the standard of care for treatment of cardiac sarcoidosis (CS). The efficacy of long‐term corticosteroid‐sparing immunosuppression in CS is unknown. The goal of this study was to assess the efficacy of methotrexate with or without adalimumab for long‐term disease suppression in CS, and to assess recurrence and adverse event rates after immunosuppression discontinuation. Methods and Results Retrospective chart review identified treatment‐naive CS patients at a single academic medical center who received corticosteroid‐sparing maintenance therapy. Demographics, cardiac uptake of 18‐fluorodeoxyglucose, and adverse cardiac events were compared before and during treatment and between those with persistent or interrupted immunosuppression. Twenty‐eight CS patients were followed for a mean 4.1 (SD 1.5) years. Twenty‐five patients received 4 to 8 weeks of high‐dose prednisone (>30 mg/day), followed by taper and maintenance therapy with methotrexate±low‐dose prednisone (low‐dose prednisone, <10 mg/day). Adalimumab was added in 19 patients with persistently active CS or in those with intolerance to methotrexate. Methotrexate±low‐dose prednisone resulted in initial reduction (88%) or elimination (60%) of 18‐fluorodeoxyglucose uptake, and patients receiving adalimumab‐containing regimens experienced improved (84%) or resolved (63%) 18‐fluorodeoxyglucose uptake. Radiologic relapse occurred in 8 of 9 patients after immunosuppression cessation, 4 patients on methotrexate‐containing regimens, and in no patients on adalimumab‐containing regimens. Conclusions Corticosteroid‐sparing regimens containing methotrexate with or without adalimumab is an effective maintenance therapy in patients after an initial response is confirmed. Disease recurrence in patients on and off immunosuppression support need for ongoing radiologic surveillance regardless of immunosuppression regimen.
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spelling doaj.art-09fd97b1b98040e68be6b46ddabcdac02022-12-21T18:09:43ZengWileyJournal of the American Heart Association: Cardiovascular and Cerebrovascular Disease2047-99802019-09-0181810.1161/JAHA.118.010952Long‐Term Corticosteroid‐Sparing Immunosuppression for Cardiac SarcoidosisDavid G. Rosenthal0Purvi Parwani1Tyler O. Murray2Bradley J. Petek3Bryan S. Benn4Teresa De Marco5Edward P. Gerstenfeld6Munir Janmohamed7Liviu Klein8Byron K. Lee9Joshua D. Moss10Melvin M. Scheinman11Henry H. Hsia12Van Selby13Laura L. Koth14Miguel H. Pampaloni15Julie Zikherman16Vasanth Vedantham17Division of Cardiology Department of Medicine University of California, San Francisco San Francisco CADivision of Cardiology Department of Medicine University of California, San Francisco San Francisco CADivision of Cardiology Department of Medicine University of California, San Francisco San Francisco CADepartment of Medicine Massachusetts General Hospital Boston MADivision of Pulmonary and Critical Care Department of Medicine University of California, San Francisco San Francisco CADivision of Cardiology Department of Medicine University of California, San Francisco San Francisco CADivision of Cardiology Department of Medicine University of California, San Francisco San Francisco CADivision of Cardiology Department of Medicine University of California, San Francisco San Francisco CADivision of Cardiology Department of Medicine University of California, San Francisco San Francisco CADivision of Cardiology Department of Medicine University of California, San Francisco San Francisco CADivision of Cardiology Department of Medicine University of California, San Francisco San Francisco CADivision of Cardiology Department of Medicine University of California, San Francisco San Francisco CADivision of Cardiology Department of Medicine University of California, San Francisco San Francisco CADivision of Cardiology Department of Medicine University of California, San Francisco San Francisco CADivision of Pulmonary and Critical Care Department of Medicine University of California, San Francisco San Francisco CADivision of Nuclear Medicine Department of Radiology University of California, San Francisco San Francisco CADivision of Rheumatology Department of Medicine University of California, San Francisco San Francisco CADivision of Cardiology Department of Medicine University of California, San Francisco San Francisco CABackground Long‐term corticosteroid therapy is the standard of care for treatment of cardiac sarcoidosis (CS). The efficacy of long‐term corticosteroid‐sparing immunosuppression in CS is unknown. The goal of this study was to assess the efficacy of methotrexate with or without adalimumab for long‐term disease suppression in CS, and to assess recurrence and adverse event rates after immunosuppression discontinuation. Methods and Results Retrospective chart review identified treatment‐naive CS patients at a single academic medical center who received corticosteroid‐sparing maintenance therapy. Demographics, cardiac uptake of 18‐fluorodeoxyglucose, and adverse cardiac events were compared before and during treatment and between those with persistent or interrupted immunosuppression. Twenty‐eight CS patients were followed for a mean 4.1 (SD 1.5) years. Twenty‐five patients received 4 to 8 weeks of high‐dose prednisone (>30 mg/day), followed by taper and maintenance therapy with methotrexate±low‐dose prednisone (low‐dose prednisone, <10 mg/day). Adalimumab was added in 19 patients with persistently active CS or in those with intolerance to methotrexate. Methotrexate±low‐dose prednisone resulted in initial reduction (88%) or elimination (60%) of 18‐fluorodeoxyglucose uptake, and patients receiving adalimumab‐containing regimens experienced improved (84%) or resolved (63%) 18‐fluorodeoxyglucose uptake. Radiologic relapse occurred in 8 of 9 patients after immunosuppression cessation, 4 patients on methotrexate‐containing regimens, and in no patients on adalimumab‐containing regimens. Conclusions Corticosteroid‐sparing regimens containing methotrexate with or without adalimumab is an effective maintenance therapy in patients after an initial response is confirmed. Disease recurrence in patients on and off immunosuppression support need for ongoing radiologic surveillance regardless of immunosuppression regimen.https://www.ahajournals.org/doi/10.1161/JAHA.118.010952immunosuppressionsarcoidosisventricular arrhythmia
spellingShingle David G. Rosenthal
Purvi Parwani
Tyler O. Murray
Bradley J. Petek
Bryan S. Benn
Teresa De Marco
Edward P. Gerstenfeld
Munir Janmohamed
Liviu Klein
Byron K. Lee
Joshua D. Moss
Melvin M. Scheinman
Henry H. Hsia
Van Selby
Laura L. Koth
Miguel H. Pampaloni
Julie Zikherman
Vasanth Vedantham
Long‐Term Corticosteroid‐Sparing Immunosuppression for Cardiac Sarcoidosis
Journal of the American Heart Association: Cardiovascular and Cerebrovascular Disease
immunosuppression
sarcoidosis
ventricular arrhythmia
title Long‐Term Corticosteroid‐Sparing Immunosuppression for Cardiac Sarcoidosis
title_full Long‐Term Corticosteroid‐Sparing Immunosuppression for Cardiac Sarcoidosis
title_fullStr Long‐Term Corticosteroid‐Sparing Immunosuppression for Cardiac Sarcoidosis
title_full_unstemmed Long‐Term Corticosteroid‐Sparing Immunosuppression for Cardiac Sarcoidosis
title_short Long‐Term Corticosteroid‐Sparing Immunosuppression for Cardiac Sarcoidosis
title_sort long term corticosteroid sparing immunosuppression for cardiac sarcoidosis
topic immunosuppression
sarcoidosis
ventricular arrhythmia
url https://www.ahajournals.org/doi/10.1161/JAHA.118.010952
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