Diagnosis and Symptomatic Treatment of Early Reactive Cardiac Amyloidosis in Systemic Sclerosis

Rae-Seok Lee; Hyun-Jin Sung; Jung Im Jung; Hea Ok Jung; Seung-Min Jung; Jennifer Jooha Lee; Seung-Ki Kwok; Ji Hyeon Ju; Sung-Hwan Park

doi:10.4078/jrd.2015.22.2.132

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Lee, Sung, Jung, Jung, Jung, Lee, Kwok, Ju, and Park: Diagnosis and Symptomatic Treatment of Early Reactive Cardiac Amyloidosis in Systemic Sclerosis

Case Report

J Rheum Dis 2015;22(2):132-136.

Published online: 31 October 2015

DOI: https://doi.org/10.4078/jrd.2015.22.2.132

Diagnosis and Symptomatic Treatment of Early Reactive Cardiac Amyloidosis in Systemic Sclerosis

Rae-Seok Lee², Hyun-Jin Sung², Jung Im Jung³, Hea Ok Jung², Seung-Min Jung^1,², Jennifer Jooha Lee^1,², Seung-Ki Kwok^1,², Ji Hyeon Ju^1,², Sung-Hwan Park^1,²

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

²Department of Internal Medicine, The Catholic University of Korea, Seoul St. Mary's Hospital, Seoul, Korea

³Department of Radiology, The Catholic University of Korea, Seoul St. Mary's Hospital, Seoul, Korea

Corresponding to: Sung-Hwan Park, Division of Rheumatology, Department of Internal Medicine, The Catholic University of Korea, Seoul St. Mary's Hospital, 222 Banpo-daero, Seocho-gu, Seoul 137-701, Korea. E-mail: rapark@catholic.ac.kr

Received 9 April 2014 Revised 20 June 2014 Accepted 22 June 2014

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

Systemic sclerosis as a connective tissue disease could affect all internal organs of the body and could also manifest as a cutaneous lesion. Cardiac involvement leading to cardiac manifestations in systemic sclerosis patients is not rare. However, cardiac amyloidosis combined with systemic sclerosis is extremely rare. Although there were no definite treatment options in this case, symptomatic treatment is the cornerstone of the management plan. In this case report, we described a correct diagnosis and symptomatic medical care of early reactive cardiac amyloidosis with systemic sclerosis and summarize the current state of the relevant literature.

Keywords: Systemic scleroderma, Restrictive cardiomyopathy, Cardiac amyloidosis

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Figure 1.

Echocardiography findings. Echocardiography shows global hypokinesia on left ventricle wall with concentric left ventricle hypertrophy (A), and E wave dominant mitral filling pattern with rapid deceleration time suggests restrictive physiology (B∼ D).

Figure 2.

Radiologic findings of magnetic resonance imaging (MRI) of the heart. Cardiac MRI with cine image shows poor contraction of left ventricle during systolic (A) and diastolic phase (B). Note diffuse wall thickening and mild dilatation of left ventricle.

Figure 3.

Cardiac biopsy specimen findings. (A) Biopsy revealing hypertrophied myocardium and interstitial edema (H&E, ×200). (B) It shows focal amyloid depositions on Congo red staining (×200), and (C) under polarized light.

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