Journal List > J Rheum Dis > v.22(2) > 1064180

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

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.

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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).
jrd-22-132f1.tif
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.
jrd-22-132f2.tif
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.
jrd-22-132f3.tif
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