Coexistence of AA and AL Cardiac Amyloidosis in a Patient with Ankylosing Spondylitis Accompanying Monoclonal Gammopathy of Undetermined Significance

Woohyeon Kim; Seon A Kim; Kyung Jin Yun; Soo Jin Na; Ji In Hyun; Jung Im Jung; Seung-Ki Kwok; Sung-Hwan Park

doi:10.4078/jrd.2014.21.3.151

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Kim, Kim, Yun, Na, Hyun, Jung, Kwok, and Park: Coexistence of AA and AL Cardiac Amyloidosis in a Patient with Ankylosing Spondylitis Accompanying Monoclonal Gammopathy of Undetermined Significance

Case Report

J Rheum Dis 2014;21(3):151-155.

Published online: 31 October 2014

DOI: https://doi.org/10.4078/jrd.2014.21.3.151

Coexistence of AA and AL Cardiac Amyloidosis in a Patient with Ankylosing Spondylitis Accompanying Monoclonal Gammopathy of Undetermined Significance

Woohyeon Kim¹, Seon A Kim¹, Kyung Jin Yun¹, Soo Jin Na¹, Ji In Hyun¹, Jung Im Jung², Seung-Ki Kwok¹, Sung-Hwan Park¹

¹Division of Rheumatology, Department of Internal Medicine, Seoul St. Mary's Hospital, College of Medicine, The Catholic University of Korea, Seoul, Korea

²Division of Rheumatology, Department of Radiology, Seoul St. Mary's Hospital, College of Medicine, The Catholic University of Korea, Seoul, Korea

Corresponding to: Seung-Ki Kwok, Division of Rheumatology, Department of Internal Medicine, Seoul St. Mary's Hospital, College of Medicine, The Catholic University of Korea, 505, Banpo-dong, Seocho-gu, Seoul 137-040, Korea. E-mail: seungki73@catholic.ac.kr

Received 20 May 2013 Revised 23 June 2013 Accepted 1 July 2013

This is a Free Access article, which permits unrestricted non-commerical use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

Amyloidosis is a clinical disorder caused by extracellular deposition of proteinaceous insoluble fibrils in various tissues, resulting in organ compromise. Amyloid L (AL) amyloidosis is the most common type of systemic amyloidosis, which occurs in association with multiple myeloma or monoclonal gammopathy of undetermined significance (MGUS). Secondary amyloid A (AA) amyloidosis is a complication of chronic inflammatory conditions, such as rheumatoid arthritis or ankylosing spondylitis. We report a case of a 49-year-old manwith a 11-year history of ankylosing spondylitis, who was recently diagnosed with MGUS presented with cardiac amyloidosis of both the AA and AL types. We report this case along with a review of relevant literature.

Keywords: Cardiac amyloidosis, Ankylosing spondylitis, Monoclonal gammopathy of undetermined significance

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Figure 1.

Lumbar radiography shows squaring of the body with the syndesmophyte along the disc margin of the lumbar spine. Facet anklyosis of the lower lumbar spine.

Figure 2.

Echocardiography demonstrated a ground glass opacity pattern of the myocardium and increased wall thickness (septal thickness, 15.9 mm; posterior wall thickness, 14.4 mm).

Figure 3.

Midventricular short-axis LGE-CMR image shows global subendocardial LGE of the left ventricle and patchy transmural enhancement of right ventricle (arrow). LGE: late gadolinium enhancement, CMR: cardiac magnetic resonance.

Figure 4.

Immunohistochemical staining of the heart biopsy showing amyloid A (AA) amyloidosis with amyloid L (AL) amylodosis. (A) AA protein (×200). (B) Lambda light chain (×200).

Figure 5.

Electron microscope revealed interstitial deposition of amorphous material with thin fibrils, consistent with amyloidosis.

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