Christine S. Siegismund1, Felicitas Escher2,3,1, Dirk Laßner1, Uwe Kühl2,1, Ulrich Gross1, Friedrich Fruhwald4, Philip Wenzel5, Thomas Münzel5, Norbert Frey6, Reinhold P. Linke7, Heinz‐Peter Schultheiß1
1Institute for Cardiac Diagnostics and Therapy (IKDT), Berlin, Germany
2Department of Cardiology, Charité Universitätsmedizin Berlin, Berlin, Germany
3Deutsches Zentrum für Herz Kreislaufforschung (DZHK), Berlin, Germany
4Division of Cardiology, Department of Internal Medicine, Medical University Graz, Graz, Austria
5Center for Cardiology - Cardiology I and Center for Thrombosis and Hemostasis University Medical Center Mainz Mainz Germany
6Department of Internal Medicine III - Cardiology, Angiology and Intensive Care Medicine University Hospital Schleswig-Holstein Kiel Germany
7amYmed, Reference Center of Amyloid Diseases Martinsried Germany
Tóm tắt
AimsTo 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 resultsWe 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).ConclusionOur 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.
