We appreciate Bodez D et al's 1 comments regarding our case of the month of a 44‐year‐old female with familial Mediterranean fever (FMF), cardiomyopathy and end stage renal disease (ESRD) 2. The patient presented with the clinical diagnoses of FMF, end stage renal disease and amyloid cardiomyopathy based on an endomyocardial biopsy demonstrating cardiac amyloidosis with vascular, perivascular and interstitial deposition of amyloid. Details of the heart examination were not included as we wanted to focus on neuropathological findings, but briefly, the heart was heavy for her height (340 grams) and demonstrated extensive amyloid deposition, positive for amyloid A, including involvement of the conduction system, predominantly in a vascular and perivascular distribution. There was moderate atherosclerosis of the coronary arteries, and mural thrombi were seen in both right and left ventricles. Although clinically significant cardiomyopathy with AA amyloidosis is uncommon, it may still be important to consider in a relatively young female with longstanding history of an inflammatory disorder with cardiac dysfunction and ESRD.
REFERENCES
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