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Year : 2018  |  Volume : 19  |  Issue : 3  |  Page : 102-105  

Full-blown rheumatoid heart and vessels associated with rheumatoid arthritis

Division of Cardiology, Department of Internal Medicine, Keimyung University Dongsan Medical Center, Daegu, Korea

Date of Web Publication18-Mar-2019

Correspondence Address:
Prof. Hyungseop Kim
Division of Cardiology, Department of Internal Medicine, Keimyung University Dongsan Medical Center, 56 Dalseong-Ro, Jung-Gu, Daegu 41931
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Source of Support: None, Conflict of Interest: None


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We report the case of a 54-year-old female patient with a history of rheumatoid arthritis (RA) who presented with dyspnea on exertion. Her pulsation on both arms was weak. Magnetic resonance (MR) angiography revealed Takayasu's arteritis (TA) characterized by multiple stenosis involving innominate, left common carotid, and left subclavian arteries. With regard to cardiac imaging, coronary computed tomography angiography revealed calcified stenosis of the left anterior descending and diagonal branch arteries, with an abdominal aorta narrowed concomitantly. Restrictive diastolic dysfunction with concentric hypertrophic myocardium was observed on echocardiography, and cardiac MR imaging revealed diffuse, subendocardial late gadolinium enhancement compatible with cardiac amyloidosis (CA). Herein, we describe a case of RA with “full-blown” complications of TA, CA, and coronary artery disease.

Keywords: Cardiac amyloidosis, coronary artery disease, rheumatoid arthritis, Takayasu's arteritis

How to cite this article:
Kim H, Kim IC. Full-blown rheumatoid heart and vessels associated with rheumatoid arthritis. Heart Views 2018;19:102-5

How to cite this URL:
Kim H, Kim IC. Full-blown rheumatoid heart and vessels associated with rheumatoid arthritis. Heart Views [serial online] 2018 [cited 2022 Nov 29];19:102-5. Available from: https://www.heartviews.org/text.asp?2018/19/3/102/254344

   Introduction Top

Rheumatoid arthritis (RA) is a progressive disorder, and its complication is related with the control of its activity or inflammation which also contributes to other vasculitis infrequently. We report a patient with long-standing RA with full-blown complication of rheumatoid heart and vessels, despite a well-controlled RA activity.

   Case Presentation Top

A 54-year-old woman presented to the cardiovascular (CV) outpatient clinic with dyspnea on exertion aggravated for 1–2 months. In addition, low systolic blood pressure (up to 60 mmHg) in both upper arms was one of the referred reasons. She was followed up for RA for 24 years and had regular medical treatment at a nearby rheumatology clinic.

On physical examination, she was alert and looked well despite the low blood pressure on her upper arms. However, the blood pressure in both lower extremities was appropriate (up to 160 mmHg). Mild pitting edema on both legs and puffy face were also noted. With regard to the upper extremities, a rheumatoid nodule was found on the right wrist and both hands were deformed and deviated to the ulnar side, compatible with RA.

Serum biochemical profile was unremarkable, while renal dysfunction (creatinine level, 2.5 mg/dL) with proteinuria was observed. The serological markers of RA were characterized with the following values: anticyclic citrullinated peptides, 150.6 U/mL; RA factor, 73.8 IU/mL; and antinuclear antibody, 1:40 (homogeneous type). Erythrocyte sedimentation rate, 32 mm/h; C-reactive protein level, 0.2 mg/dL.

She was controlled with broad-spectrum medications, including methotrexate, methylprednisolone, hydroxychloroquine, sulfasalazine, and nonsteroid anti-inflammatory drugs (NSAIDs).

Electrocardiography revealed normal sinus rhythm with a left ventricular (LV) hypertrophic pattern. Echocardiographic study revealed global hypokinesia with reduced ejection fraction (35%) and LV concentric hypertrophy with apical aneurysm, in which multiple, small thrombi were embedded [Figure 1]. Mitral inflow showed a restrictive pattern (E/A ratio, 3.6; isovolumic relaxation time, 50 ms; deceleration time, 130 ms; E/e' ratio, 21.0).
Figure 1: Restrictive cardiomyopathy demonstrated by echocardiographic study. A thickened myocardium with apical thrombus was observed in apical four-chamber and parasternal short-axis view (a and b), and diastolic dysfunction of restrictive pattern was revealed by mitral inflow and tissue Doppler velocity patterns (c and d): high early-/low late-mitral inflow velocities (E/A ratio: 3.6), short deceleration (130 ms), and isovolumic relaxation times and low septal early-/late-diastolic velocities (arrows: e' velocity of 6.2 cm/s)

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Coronary computed tomography angiography revealed 70%–80% stenosis of the proximal left anterior descending artery and 70% stenosis of the diagonal branch and luminal narrowing of the descending abdominal aorta [Figure 2]. Cardiac magnetic resonance (MR) imaging revealed diffuse, circular, and patched late gadolinium enhancements in the hypertrophied myocardium [Figure 3]a, [Figure 3]b, [Figure 3]c, [Figure 3]d. It is interesting that the best inversion time (TI) for myocardial nulling was greatly shortened (120 ms) on the TI-scout sequence. These findings were also consistent with cardiac amyloidosis (CA). The serum levels of free kappa and lambda light chains measured to rule out AL-type CA were within their normal ranges. MR angiography revealed total occlusion of both the subclavian and carotid arteries in the proximal portion, with multiple collateral flow to the upper neck and head side [Figure 3]e.
Figure 2: Coronary computed tomography showed tight calcified stenosis of the proximal left anterior descending artery (arrowheads) (a) with its diagonal branch (arrowheads) (b) and with apical mural thrombus (asterisk) in the left ventricular cavity in two-chamber view (c). The narrowed abdominal aortic lumen (arrows) was observed in sagittal view of abdominal computed tomography (d)

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Figure 3: Systemic cardiac amyloidosis demonstrated by cardiac magnetic resonance imaging (a-d). Cardiac magnetic resonance showing small amount of pericardial effusion and thickened myocardium in short-axis view. Through a-d, diffuse, circular, subendocardial, or patched late gadolinium enhancement is typically observed. Head/neck magnetic resonance angiography showed near-total occlusion of innominate (arrows), left common carotid (arrowhead), and left subclavian arteries (asterisk) with collateral small vessels to cerebral supply (e)

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According to CV imaging, the final diagnosis was RA associated with Takayasu's arteritis (TA), reactive CA, and ischemic heart disease with coronary artery stenosis. Anticoagulation cleared the apical thrombus from the LV cavity, and medications for ischemic treatment were maintained. However, she refused surgery for revascularization of the carotid, subclavian, and coronary arteries and opted for medical treatment with regular follow-up.

   Discussion Top

RA is a chronic progressive immunological disorder that primarily involves the musculoskeletal system. The complications of RA are related with the disease activity and duration. Thus, the inflammation of RA should be controlled and suppressed using NSAIDs, steroids, or disease-modifying antirheumatic drugs (or immunosuppressive agents). Long-standing RA could, though rarely, cause complications of TA and other associated vasculitis.[1],[2] The chronic progression of inflammation by RA would be associated with TA. However, considering her medical history and normal C-reactive protein level, the disease activity had been under good control.

The patient showed ischemic heart disease caused by coronary artery stenosis with LV apical thrombus. TA per se could involve the coronary artery in addition to the aorta. Furthermore, systemic CA has been associated with RA, irrespective of TA, and is thought to be a poor prognostic factor in systemic disorders.[3] In contrast to AL-type CA, systemic CA seems rare. In the present case, the serum levels of free kappa/lambda light chains were normal, which indicates another reactive CA type, not AL-type CA. Hence, in the present study, we found that chronic systemic disease is required for AA-type CA. Although the definite diagnosis requires tissue biopsy demonstrating amyloid infiltration, it is not easy, with high rates of false-negative findings. Therefore, obtaining cardiac MR findings could be an alternative method for cardiac tissue characterization.[4]

The time to diagnosis of CV complications from the first diagnosis of RA may differ, but it usually requires a longer duration (16–35 years) for CA cases[5],[6] and a relatively short duration (1–15 years) for TA cases[1],[2] in the literature. In our case, the required time duration was >20 years for major CA symptoms. Considering this duration for CV manifestation of RA complication, CA as a poor prognostic indicator would be one of the last terminal destinations of complications.

   Conclusion Top

Simultaneous full-blown CV complications have not been reported yet in a single patient. Major complications of TA, coronary artery disease, and systemic CA were manifested in a patient with long-standing RA, even with a well-controlled RA activity. Therefore, watchful evaluation for late RA complications is necessary.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient has given her consent for her images and other clinical information to be reported in the journal. The patient understand that her name and initial will not be published and due efforts will be made to conceal her identity, but anonymity cannot be guaranteed.

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Conflicts of interest

There are no conflicts of interest.

   References Top

Wada Y, Kobayashi D, Murakami S, Oda M, Hanawa H, Kuroda T, et al. Cardiac AA amyloidosis in a patient with rheumatoid arthritis and systemic sclerosis: The therapeutic potential of biological reagents. Scand J Rheumatol 2011;40:402-4.  Back to cited text no. 1
Verweij KE, van Well AM, Vd Sluijs JW, Dees A. Late onset Takayasu arteritis and rheumatoid arthritis. Case Rep Med 2012;2012:523218.  Back to cited text no. 2
Korkmaz C, Zubaroǧlu I, Kaya T, Akay OM. Takayasu's arteritis associated with rheumatoid arthritis: A case report and review of the literature. Rheumatology (Oxford) 2001;40:1420-2.  Back to cited text no. 3
Ben Abdelghani K, Fazaa A, Ben Abdelghani K, Laatar A, Khedher A, Zakraoui L, et al. Chronic inflammatory rheumatism associated with Takayasu disease. Ann Vasc Surg 2013;27:353.e1-6.  Back to cited text no. 4
Goeminne A, Missault L, Bauwens F, De Pauw M, De Sutter J. Clinical experience with cardiac amyloidosis. Acta Cardiol 2003;58:143-7.  Back to cited text no. 5
Di Bella G, Pizzino F, Minutoli F, Zito C, Donato R, Dattilo G, et al. The mosaic of the cardiac amyloidosis diagnosis: Role of imaging in subtypes and stages of the disease. Eur Heart J Cardiovasc Imaging 2014;15:1307-15.  Back to cited text no. 6


  [Figure 1], [Figure 2], [Figure 3]

This article has been cited by
1 Coronary artery involvements in Takayasu arteritis: systematic review of reports
Shi-Min Yuan,Hui-Zhen Lin
General Thoracic and Cardiovascular Surgery. 2020; 68(9): 883
[Pubmed] | [DOI]


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