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Vasculitis

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Skin and the Heart

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Abstract

Vasculitis is an inflammation and destruction of the blood vessel wall. It can occur in any organ system of the body, and can present in a systemic form with multi-organ involvement, or in a limited form with only one organ system involved. The skin is one of the most frequently involved organs in vasculitis with the most frequent clinical presentation being purpura. Although symptomatic cardiac involvement is relatively rare, it is clinically important because of its prognostic value. Cardiac involvement is most common in Takayasu arteritis, polyarteritis nodosa, and eosinophilic granulomatosis with polyangiitis. Pericarditis, myocarditis, coronary arteritis, valvulopathy, and intracavitary cardiac thrombosis are the major cardiac manifestations in primary vasculitis. The diagnosis of cardiac involvement often requires high suspicion. Endomyocardial biopsy is not always possible. Echocardiography is the primary imaging modality and an important tool for the diagnosis and follow-up of cardiac involvement. Cardiac magnetic resonance imaging might be the most sensitive imaging modality for the evaluation of cardiac involvement. Treatment of cardiac involvement depends on cardiac manifestations. Treatment of myocarditis and coronary arteritis is much more aggressive than pericarditis.

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Abbreviations

AAV:

Antineutrophil cytoplasmic antibody associated vasculitis

ANCA:

Antineutrophil cytoplasmic antibodies

anti-GBM:

Anti-glomerular basement membrane

CHCC:

International Chapel Hill Consensus Conference Nomenclature system

CSVV:

Cutaneous small vessel vasculitis

CT:

Computed tomography

CTA:

Computed tomography angiography

CV:

Cryoglobulinemic vasculitis

D-CHCC:

Dermatologic Addendum to the 2012 Revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides

DIF:

Direct immunofluorescence

EED:

Erythema elevatum et diutinum

EGPA:

Eosinophilic granulomatosis with polyangiitis

FDG-PET:

Fluoro-2-deoxyglucose positron-emission tomography

GCA:

Giant cell arteritis

GPA:

Granulomatosis with polyangiitis

HBV:

Hepatitis B virus

HCV:

Hepatitis C virus

HUV:

Hypocomplementemic urticarial vasculitis

Ig:

Immunoglobulin

IgAV:

IgA vasculitis

KD:

Kawasaki disease

MPA:

Microscopic polyangiitis

MRA:

Magnetic resonance angiography

MRI:

Magnetic resonance imaging

PAN:

Polyarteritis nodosa

TAK:

Takayasu arteritis

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Authors and Affiliations

  1. Clinic of Dermatology and Venereology, Adıyaman University Training and Research Hospital, Adıyaman, Turkey

    Birgül Özkesici Kurt

  2. Department of Dermatology and Venereology, Akdeniz University School of Medicine, Antalya, Turkey

    Erkan Alpsoy

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  1. Birgül Özkesici Kurt
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  2. Erkan Alpsoy
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Correspondence to Erkan Alpsoy .

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Editors and Affiliations

  1. Paediatric Dermatology Department, Colentina Clinical Hospital, “Carol Davila”, University of Medicine and Pharmacy, Bucharest, Romania

    Carmen Salavastru

  2. Professor of Dermatology, University of New South Wales, Sydney, NSW, Australia

    Dedee F. Murrell

  3. Department of Cardiology, Liverpool Hospital, University of New South Wales, Sydney, NSW, Australia

    James Otton

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Kurt, B.Ö., Alpsoy, E. (2021). Vasculitis. In: Salavastru, C., Murrell, D.F., Otton, J. (eds) Skin and the Heart. Springer, Cham. https://doi.org/10.1007/978-3-030-54779-0_10

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