Christine S Siegismund1, Felicitas Escher1,2,3, Dirk Lassner1, Uwe Kühl1,2, Ulrich Gross1, Friedrich Fruhwald4, Philip Wenzel5, Thomas Münzel5, Norbert Frey6, Reinhold P Linke7, Heinz-Peter Schultheiss1. 1. Institute for Cardiac Diagnostics and Therapy (IKDT), Berlin, Germany. 2. Department of Cardiology, Charité - Universitätsmedizin Berlin, Berlin, Germany. 3. Deutsches Zentrum für Herz-Kreislaufforschung (DZHK), Berlin, Germany. 4. Division of Cardiology, Department of Internal Medicine, Medical University Graz, Graz, Austria. 5. Center for Cardiology - Cardiology I and Center for Thrombosis and Hemostasis, University Medical Center Mainz, Mainz, Germany. 6. Department of Internal Medicine III - Cardiology, Angiology and Intensive Care Medicine, University Hospital Schleswig-Holstein, Kiel, Germany. 7. amYmed, Reference Center of Amyloid Diseases, Martinsried, Germany.
Abstract
AIMS: To evaluate the influence of endomyocardial biopsy (EMB)-proven intramyocardial inflammation on mortality in patients with cardiac transthyretin amyloid (ATTR) or amyloid light-chain (AL) amyloidosis. METHODS AND RESULTS: We included 54 consecutive patients (mean age 68.83 ± 9.59 years; 45 men) with EMB-proven cardiac amyloidosis. We followed up patients from first diagnostic biopsy to as long as 36 months (mean 11.5 ± 12 months) and compared their outcome with information on all-cause mortality with or without proof of inflammation on EMB. Intramyocardial inflammation was assessed by quantitative immunohistology. Patients suffering from amyloidosis revealed a significant poor prognosis with proof of intramyocardial inflammation in contrast to those without inflammation (log-rank P = 0.019). Re-grouping of patients indicated AL amyloidosis to have a significant impact on all-cause mortality (log-rank P = 0.012). The detailed subgroup analysis showed that patients suffering from AL amyloidosis with intramyocardial inflammation have a significantly worse prognosis compared with AL amyloidosis without inflammation and ATTR with or without inflammation, respectively (log-rank P = 0.014, contingency Fisher's exact test, P = 0.008). CONCLUSION: Our study reports for the first time a high incidence (48.1%) of intramyocardial inflammation in a series of patients with EMB-proven cardiac amyloidosis and could show that in patients with AL amyloidosis, intramyocardial inflammation correlated significantly with increased mortality. Our data have a direct clinical impact because one can hypothesize that additional immunomodulating/anti-inflammatory treatment regimens in patients with biopsy-proven inflammation of heart muscle tissue could be beneficial for patients suffering from cardiac AL amyloidosis.
AIMS: To evaluate the influence of endomyocardial biopsy (EMB)-proven intramyocardial inflammation on mortality in patients with cardiac transthyretin amyloid (ATTR) or amyloid light-chain (AL) amyloidosis. METHODS AND RESULTS: We included 54 consecutive patients (mean age 68.83 ± 9.59 years; 45 men) with EMB-proven cardiac amyloidosis. We followed up patients from first diagnostic biopsy to as long as 36 months (mean 11.5 ± 12 months) and compared their outcome with information on all-cause mortality with or without proof of inflammation on EMB. Intramyocardial inflammation was assessed by quantitative immunohistology. Patients suffering from amyloidosis revealed a significant poor prognosis with proof of intramyocardial inflammation in contrast to those without inflammation (log-rank P = 0.019). Re-grouping of patients indicated AL amyloidosis to have a significant impact on all-cause mortality (log-rank P = 0.012). The detailed subgroup analysis showed that patients suffering from AL amyloidosis with intramyocardial inflammation have a significantly worse prognosis compared with AL amyloidosis without inflammation and ATTR with or without inflammation, respectively (log-rank P = 0.014, contingency Fisher's exact test, P = 0.008). CONCLUSION: Our study reports for the first time a high incidence (48.1%) of intramyocardial inflammation in a series of patients with EMB-proven cardiac amyloidosis and could show that in patients with AL amyloidosis, intramyocardial inflammation correlated significantly with increased mortality. Our data have a direct clinical impact because one can hypothesize that additional immunomodulating/anti-inflammatory treatment regimens in patients with biopsy-proven inflammation of heart muscle tissue could be beneficial for patients suffering from cardiac AL amyloidosis.
Authors: Selina J Hein; Maximilian Knoll; Fabian Aus dem Siepen; Jennifer Furkel; Stefan Schoenland; Ute Hegenbart; Hugo A Katus; Arnt V Kristen; Mathias Konstandin Journal: World J Cardiol Date: 2021-03-26
Authors: Kathleen W Zhang; Jennifer Miao; Joshua D Mitchell; Jose Alvarez-Cardona; Kelsey Tomasek; Yan Ru Su; Mary Gordon; R Frank Cornell; Daniel J Lenihan Journal: JACC CardioOncol Date: 2020-03-17