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.

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

REFERENCES

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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.

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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).

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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.

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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).

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Figure 5.

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

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