Specific cardiomyopathy in lupus patients: report of three cases

doi:10.1016/S1297-319X(03)00101-5

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Volume 71, Issue 1, January 2004, Pages 66-69

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Abstract

Clinically important myocarditis is an unusual feature in patients with systemic lupus erythematosus (SLE). We report three consecutive lupus patients over a 1 year period who developed severe left ventricular dysfunction in the absence of coronary artery disease or hypertensive cardiomyopathy. Two of them had clinical and biological flare of the disease whereas the lupus was quiescent in the latter. Two of them had positive IgG anticardiolipin antibodies. High dose steroids were given in two patients; one of them also required cyclophosphamide on account of diffuse proliferative glomerulonephritis. Left ventricular function improved quickly and markedly in these two patients; one of them had recurrence of severe myocarditis at intervals of 6 years and was each time responsive to steroids. Lupus cardiomyopathy, a rare event in the course of SLE, can be related to the disease even in the absence of coronary artery disease or hypertensive cardiomyopathy. It may be improved by steroids and immunosuppressive therapy. Literature concerning this cardiac manifestation in lupus is reviewed.

Introduction

Cardiac involvement in systemic lupus erythematosus (SLE) is well known. Cardiac manifestations of SLE include pericarditis, myocarditis, valvular disease, ischemic coronary artery disease, conduction abnormalities, arrhythmias. Although myocardial abnormalities are frequent in autopsic studies, clinically patent myocarditis occurs in less than 10% of patients [1]. We describe three patients with SLE seen over a 1 year period, who presented with rapid onset of left ventricular dysfunction (Table 1).

Section snippets

Patient 1

A 43-year-old woman with a history of longstanding Raynaud’s phenomenon presented with polyarthralgias and myalgias. Initial examination showed synovitis in both wrists and fingertips.

Laboratory tests revealed a strongly positive antinuclear antibody (ANA) at 1:1280 with presence of anti-Sm and anti-RNP antibodies. Anti-dsDNA were negative. Signs of vasculitis were present on muscular biopsy. SLE was diagnosed and treatment with prednisone 20 mg per day and hydroxychloroquine 400 mg per day was

Discussion

Severe myocardial dysfunctions are uncommon during SLE, and most often associated with coronary atherosclerosis, longstanding hypertension and corticotherapy. We report three cases of cardiomyopathy with congestive heart failure, in the absence of hypertensive coronary artery disease, characterized by their early occurrence in the course of the lupus disease. Two of these cases, who were treated with high-dose corticotherapy had a significant improvement of the left ventricular function. The

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Dilated cardiomyopathy complicated by an intracavitary thrombus and acute heart failure: A rare presentation of systemic lupus erythematosus
2022, Annals of Medicine and Surgery
Citation Excerpt :
The exact pathogenesis of myocardial involvement is not entirely understood [13]. Case reports of SLE patients with cardiac manifestations who had undergone myocardial biopsy lack evidence for myocarditis [3,14]. This suggest that myocardial inflammation may not be the pathophysiological basis, and that other factors such as thrombotic or inflammatory microvascular coronary disease may play a role [15].
Cardiac manifestations during systemic lupus erythematosus (SLE) are diverse and often have major prognostic consequences. Lupus cardiomyopathy is an uncommon event in the course of SLE and initial clinical manifestation as decompensated dilated cardiomyopathy is very rare.
we report the case of a 52-years-old female who presented with acute onset decompensated dilated cardiomyopathy as the initial feature of SLE. The diagnosis was based on clinical, electrocardiographic, angiographic and biochemical characteristics.
Although rare, SLE cardiomyopathy deserves the attention due to its infrequent clinical presentation. It is a complex disease that requires prompt investigation and treatment, otherwise the damage is unrecoverable.
Off-label use of intravenous immunoglobulin therapy in the treatment of lupus myocarditis: Two case reports and literature review
2017, Revue de Medecine Interne
Plusieurs cas cliniques ont rapporté le bénéfice des immunoglobulines intraveineuses (IgIV) au cours du lupus.
Nous décrivons ici deux cas de myocardite lupique traités par IgIV à dose immunomodulatrice. Le premier concerne une patiente de 42 ans qui présentait une myocardite lupique résistante au traitement par corticoïdes et cyclophosphamide. Les IgIV à la dose de 2 g/kg ont permis une amélioration clinique en quelques jours. Le second cas concerne une patiente de 43 ans qui était traitée par IgIV à la dose de 2 g/kg suite au diagnostic de myocardite et chez qui les immunosuppresseurs étaient contre-indiqués du fait du récent contexte d’endocardite infectieuse. Une amélioration clinique était observée avec une augmentation de la fraction d’éjection du ventricule gauche de 20 % à 60 % en quelques jours. Nous rapportons également neuf observations similaires issues de la littérature.
L’utilisation des IgIV dans la myocardite lupique n’est pas reconnue officiellement mais pourrait être une alternative lorsque les thérapies conventionnelles ont échoué ou sont contre-indiquées.
Several case reports have reported the benefit of intravenous immunoglobulin therapy in many autoimmune diseases, including systemic lupus erythematosus.
Here, we report on two cases of lupus myocarditis treated with high dose of intravenous immunoglobulin. The first patient was a 42-year-old woman who presented with lupus myocarditis that was resistant to corticosteroids and cyclophosphamide, and who was finally successfully treated with a single dose of 2 g/kg of intravenous immunoglobulin. The patient displayed clinical improvement a few days later. The second case – a 43-year-old woman was diagnosed with lupus myocarditis and immunosuppressive drugs were contraindicated because of the context of a recent infective endocarditis. She was treated with repeated dose of 2 g/kg of intravenous immunoglobulin. Clinical improvement was observed and the left ventricular ejection fraction increased from 20 % to 60 % within a few days. We also report 9 similar observations identified from a literature review.
The use of intravenous immunoglobulin in lupus myocarditis is not officially recognized but could be considered as an alternative when conventional therapies have failed or are contraindicated.
The use of glucocorticoids in Systemic Lupus Erythematosus. After 60years still more an art than science
2013, Autoimmunity Reviews
Citation Excerpt :
Oral prednisone dose ranges from 30 mg to 1 mg/kg in both groups with the lower dosages in the pericarditis group (about 30 mg) and higher dosage in myocarditis (about 1 mg/kg). Most publications report a high response rate (defined by normalization of ejection fraction and/or resolution of pericardial effusion) [96–111]. Treatment of pulmonary manifestations with GCs is scarcely published in the literature.
Systemic Lupus Erythematosus (SLE) is a clinically diverse, chronic autoimmune disease with inflammation in several organ systems. Its pathogenesis is complex, but includes many factors that can be influenced by glucocorticoids (GCs). Indeed, GCs constitute the corner-stone in SLE-treatment. However, guidelines for GC-treatment of the different disease manifestations are lacking and not every patient responds (sufficiently). The focus of this systematic review is to evaluate the differential glucocorticoid treatment of various SLE manifestations. In addition, some relevant mechanisms of glucocorticoid action as well as resistance are discussed.
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Citation Excerpt :
Favorable response to cyclophosphamide or azathioprine, following as a steroid sparing agent, has also been reported [97]. The administration of intravenous immunoglobulin in severely ill patients with lupus myocarditis refractory to steroids has shown good clinical outcome with rapid recovery and resolution of left ventricular abnormalities in small series of patients [98,99]. It has been suggested that IVIG prevents humoral immunity induced myocyte damage through its anti-idiotypic properties and diminishes the expression and the synthesis of TNF-alpha, IL-6 and other proinflammatory cytokines [100].
Autoimmune responses and inflammation are involved in the excess cardiovascular risk observed in patients with systemic inflammatory diseases. Autoimmune myocarditis is a presentation of an inflammatory reaction of the heart during the course of autoimmune disorders, with most cases seen in systemic lupus erythematosus. Early diagnosis is of great significance because of the likelihood of progression to severe and potentially fatal complications such as arrhythmias, heart block, and heart failure. The clinical presentation of the disease is silent leading to delayed diagnosis when dilated cardiomyopathy or heart failure has already advanced. Therefore, a major issue is whether the diagnosis of myocarditis will continue to require invasive procedures such as endomyocardial biopsy or can be achieved with non-invasive methods. There is increasing evidence that noninvasive cardiac imaging, including tissue Doppler echocardiography and cardiac magnetic resonance (CMR), is able to detect subclinical cases and aid in the initiation of specific treatment when it is more likely to be effective. CMR in particular, has emerged as an important technique in the evaluation of myocarditis using three types of images: T2-weighted (T2-W), early T1-weighted (EGE) images taken after 1 min, and delayed enhanced images (LGE) taken 15 min after the injection of contrast agent. If 2/3 of the imaging sequences are positive, myocardial inflammation can be predicted or ruled out with a diagnostic accuracy of 78%. As our understanding of disease mechanisms improves, multimodality imaging may aid in the development of new diagnostic and therapeutic strategies for this potentially devastating complication of systemic inflammation, but further studies are needed to formally evaluate this.
Dilated cardiomyopathy: An unusual and severe condition in juvenile systemic lupus erythematosus
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The spectrum of lupus myocarditis: from asymptomatic forms to cardiogenic shock
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Case reportSpecific cardiomyopathy in lupus patients: report of three cases

Abstract

Introduction

Section snippets

Patient 1

Discussion

Mayo Clin Proc

Am J M

Am J M

Am J M

Endomyocardial biopsy in patients with systemic lupus erythematosus

J Rheumatol

Case report
Specific cardiomyopathy in lupus patients: report of three cases