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Cardiac involvement in primary systemic vasculitis and potential drug therapies to reduce cardiovascular risk

  • Observational Research
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Abstract

Cardiac involvement is common in primary systemic vasculitides and may be due to direct effect of the disease on the heart or due to therapy. We shall review involvement of the heart in the various forms of primary systemic vasculitis. Among anti-neutrophil cytoplasmic antibody-associated vasculitis (AAV), eosinophilic granulomatosis with polyangiitis most commonly involves the heart. Involvement of the heart confers poorer prognosis in AAV, which is also complicated by increased risk of cardiovascular events. Kawasaki’s disease (KD) is the most common form of medium-vessel vasculitis to affect the heart, with coronary artery aneurysms being the most common manifestation. These predispose patients with KD to develop premature ischemic heart disease. Takayasu’s arteritis is the most common large-vessel vasculitis to involve the heart and can result in aortic incompetence, myocarditis, or coronary heart disease. Involvement of the heart in Behcet’s disease is usually in the form of intracardiac mass lesions, thrombosis, or endomyocardial fibrosis. Drugs used in the treatment of systemic vasculitis influence the risk of developing cardiovascular events. Corticosteroid therapy has been shown to increase the risk of myocardial infarction, whereas methotrexate, azathioprine, mycophenolate mofetil, rituximab, and anti-tumor necrosis alpha agents favorably modulate the risk of cardiovascular events, predominantly by dampening systemic inflammation. Awareness of cardiac involvement in vasculitis and accelerated cardiovascular risk in these patients should help clinicians to maximize the modulation of modifiable risk factors for heart disease in these individuals.

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Abbreviations

AAV:

ANCA-associated small-vessel vasculitis

ABA:

Abatacept

AMP:

Adenosine monophosphate

AMPK:

Adenosine monophosphate-activated protein kinase

ANCA:

Anti-neutrophil cytoplasmic antibodies

ANCA+:

Anti-neutrophil cytoplasmic antibodies positive

ANCA−:

Anti-neutrophil cytoplasmic antibodies negative

AZA:

Azathioprine

BD:

Behcet’s disease

CANTOS:

Canakinumab Anti-inflammatory Thrombosis Outcomes Study

CMR:

Cardiac magnetic resonance

CRP:

C-reactive protein

CREB:

Cyclic AMP-responsive element-binding protein

CS:

Cogan syndrome

EGPA:

Eosinophilic granulomatosis with polyangiitis

EP:

Electrophysiologic

GCA:

Giant cell arteritis

GPA:

Granulomatosis with polyangiitis

HDL:

High-density lipoproteins

IL:

Interleukin

IL1RA:

Interleukin 1 receptor antagonist

IVIG:

Intravenous immunoglobulin

KD:

Kawasaki’s disease

LDL:

Low-density lipoproteins

MCP1:

Monocyte chemoattractant protein 1

MPA:

Microscopic polyangiitis

MTX:

Methotrexate

MYCOP:

Mycophenolate

NCEP/ATP III:

National Cholesterol Education Program/Adult Treatment Panel III

PAN:

Polyarteritis nodosa

PET:

Positron emission tomography

QTc:

Corrected QT interval

RTX:

Rituximab

STE:

Speckle-tracing two-dimensional echocardiography

TA:

Takayasu’s arteritis

TG:

Triglycerides

TLR:

Toll-like receptor

TNF:

Tumor necrosis factor

TOC:

Tocilizumab

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Acknowledgments

The authors would like to acknowledge Dr Vikas Agarwal and Professor Vir Singh Negi for their encouragement and suggestions toward improvement of the manuscript.

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  1. Department of Clinical Immunology, Jawaharlal Institute of Postgraduate Medical Education and Research (JIPMER), Puducherry, 605006, India

    Durga Prasanna Misra

  2. Department of Clinical Immunology and Rheumatology, Kasturba Medical College Hospital, Mangalore, 575001, Karnataka, India

    Sajjan N. Shenoy

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Misra, D.P., Shenoy, S.N. Cardiac involvement in primary systemic vasculitis and potential drug therapies to reduce cardiovascular risk. Rheumatol Int 37, 151–167 (2017). https://doi.org/10.1007/s00296-016-3435-1

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