Fulminant lymphocytic myocarditis associated with orbital myositis and diaphragmatic paralysis

doi:10.1016/j.carpath.2015.08.004

Cardiovascular Pathology

Volume 25, Issue 1, January–February 2016, Pages 55-58

https://doi.org/10.1016/j.carpath.2015.08.004 Get rights and content

Abstract

Although the clinical presentation of myocarditis is very diverse, ranging from mild dyspnea to hemodynamic collapse, myocarditis accompanied with extracardiac myositis is extremely rare. We report a single case of fulminant myocarditis associated with orbital myositis and diaphragmatic paralysis in a 40-year-old man, which was successfully managed by immunosuppressive therapy with steroid.

Introduction

Myocarditis is typically the result of a viral infection; causative viruses include coxsackie B virus, parvovirus B19, adenovirus, influenza virus, human immunodeficiency virus (HIV), and hepatitis C virus [1]. In addition to the acute insult associated with the viral infection, postviral immune-mediated cardiac injury may cause contractile dysfunction in the subacute to chronic phase, resulting in dilated cardiomyopathy [1].

The clinical presentation of myocarditis is variable and may involve a viral prodrome with general weakness, fever, myalgia, respiratory symptoms, and gastrointestinal symptoms. Cardiac symptoms are also very diverse and range from self-limiting mild dyspnea to devastating hemodynamic collapse [1]. The coexistence of myocarditis with ocular myositis and diaphragmatic paralysis is very rare. Here, we report a single case of myocarditis which presented with binocular diplopia and severe orthopnea secondary to bilateral diaphragmatic paralysis.

Section snippets

Case

A 40-year-old man, who was otherwise well and led an active life, presented with dyspnea on the background of a 2-week history of flu-like symptoms. He received supportive therapy in another hospital, during which time his dyspnea worsened. He also developed binocular diplopia and pain in the left eye and was subsequently referred to our hospital. The patient had one episode of ventricular tachycardia which was cardioverted in previous admission. Coronary angiography, performed during the

Discussion

Myocarditis with skeletal muscle myositis is generally associated with giant cell myocarditis. Lymphocytic myocarditis with skeletal muscle myositis is a rare condition, although the literature does contain previous reports [2], [3], [4]. However, the case presented here is unique; the involved skeletal muscles (diaphragm and EOM) differ, in terms of functional and structural characteristics, to the limb muscles [5], [6] involved in previous reports [3], [4]. The other notable finding in this

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Cited by (7)

Efficacy of immunosuppressive therapy in myocarditis: A 30-year systematic review and meta analysis
2021, Autoimmunity Reviews
Citation Excerpt :
Azadeh Tajmir-Riahi et al. [42] treated with steroids one case of active lymphocytic myocarditis associated with the immune checkpoint inhibitor nivolumab, with LVEF increase from 15% to 40%. Oh Hong Kwon [43] treated one fulminant lymphocytic myocarditis case, which was associated with orbital myositis and diaphragmatic paralysis, with steroids only, with improvement of LVEF (from 19 to 45%) at 12 days. Another attempt [44] included OKT3 monoclonal antibody leading to improvement of heart failure in a patient with fulminant lymphocytic myocarditis with intractable ventricular arrhythmias.
Myocarditis is an inflammation of the heart muscle, due to infectious, toxic or autoimmune causes. Literature reported controversial results in relation to the effect of immunosuppression (IS)/immunomodulation (IM). We aimed at assessing the effect of IS/IM by meta analysis.
Using the P.R.I.S.M.A. approach, two researchers searched for relevant studies on PubMed, Embase, and the Central Registry of Controlled Trials of the Cochrane Library. Proposed MeSH terms were: “immunotherapy OR immune therapy OR immune modeling OR Immunosuppressive Agents” AND “combination OR combined with OR plus” AND “myocarditis OR cardiomyopathies OR inflammatory cardiomyopathy”. The language was restricted to English. Reference lists of included articles and those relevant to the topic were hand searched for the identification of additional, potentially relevant articles. The cutoff date was from 1987 until 30th Nov 2019. Reported survival or mortality events or change of left ventricular ejection fraction (LVEF) after IS/IT were primary outcomes of the study; in addition, improvement of New York Heart Association class, follow-up biopsy (Bx) findings, viral genome clearance on Bx and recurrence of myocarditis were recorded if reported. Statistical analysis was conducted using Review Manager 5.3; 5452 studies were screened, of these 73 were assessed for eligibility, including 8 randomized control studies, 26 retrospective studies, 2 prospective studies and 1 case control study, 34 case reports and 2 case series. In prospective studies, the difference in mortality between the IS and control groups tended to be lower in the combined IS groups (12.5% vs. 18.2%) (95% CI of odds ratio 0.7(0.3, 1.64)) and the pooled difference of the increase of LVEF between the IS and control groups tended to be higher in the combined IS groups (95% CI 7.26 (−2.29, 16.81)). In retrospective studies, the difference of survival between the IS and control group was significantly in favor of IS (95%CI Hazard ratio 0.82(0.69, 0.96)). Conclusions. A tailored IS may be considered in myocarditis, depending on the phase of the disease, and the type of underlying autoimmune or immune-mediated form.
Clinical Manifestations of and Laboratory Tests for Myocarditis and Fulminant Myocarditis
2022, Fulminant Myocarditis
Orbital giant cell myositis is an unusual and potentially lethal cause of bilateral ophthalmoplegia–A case report and literature review
2022, Orbit (London)
Clinical characteristics, time course, treatment and outcomes of patients with immune checkpoint inhibitor-associated myocarditis
2021, Journal for ImmunoTherapy of Cancer
Orbital Myositis: A Comprehensive Review and Reclassification
2020, Ophthalmic Plastic and Reconstructive Surgery
Acute and Fulminant Myocarditis: a Pragmatic Clinical Approach to Diagnosis and Treatment
2018, Current Cardiology Reports

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: Conflict of interest: None

: All authors of this case did not take financial supports.

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Clinical Case ReportFulminant lymphocytic myocarditis associated with orbital myositis and diaphragmatic paralysis

Abstract

Introduction

Section snippets

Case

Discussion

J Am Coll Cardiol

Can J Cardiol

Can J Cardiol

J Neurol Sci

Crit Care Clin

Clinical Case Report
Fulminant lymphocytic myocarditis associated with orbital myositis and diaphragmatic paralysis