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Vasculitis

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Rheumatology for Primary Care Providers

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Abstract

The vasculitides are a rare group of heterogeneous diseases where the defining feature is inflammation of vessel walls, leading to end-organ damage. These conditions have been usually classified based on the caliber of the blood vessel involved. In recent years, our understanding of these conditions has increased, leading to the development of new therapies and better outcomes in their treatment. In this chapter, we summarize the clinical presentation as well as the diagnostic approach and treatment to the most common forms of primary systemic vasculitis.

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Abbreviations

AAV:

ANCA-associated vasculitis

ADA2:

Adenosine deaminase 2

AION:

Acute ischemic optic neuropathy

ANCA:

Antineutrophil cytoplasmic antibody

BS:

Behcet’s syndrome

c-ANCA:

cytoplasmic ANCA

CNS:

Central nervous system

CRP:

C-reactive protein

CTA:

Computed tomography angiography

CTD:

Connective tissue diseases

CV:

Cryoglobulinemic vasculitis

DAH:

Diffuse alveolar hemorrhage

ECG:

Electrocardiogram

EGPA:

Eosinophilic granulomatosis with polyangiitis

ESR:

Erythrocyte sedimentation rate

FDG-PET:

18-fluorodeoxyglucose-positron tomography

GBM:

Anti-glomerular basement membrane

GCA:

Giant cell arteritis

GCs:

Glucocorticoids

GI:

Gastrointestinal

GPA:

Granulomatosis with polyangiitis

HBV:

Hepatitis B virus

HCV:

Hepatitis C virus

HLA:

Human leukocyte antigen

ICI:

Immune-checkpoint inhibitors

IRAE:

Immune-related adverse event

LVV:

Large-vessel vasculitis

MPA:

Microscopic polyangiitis

MPO:

Myeloperoxidase

MRA:

Magnetic resonance angiography

MRI:

Magnetic resonance imaging

PAN:

Polyarteritis nodosa

p-ANCA:

peripheral ANCA

PFAPA:

Periodic fever, aphthous stomatitis, pharyngitis, and cervical adenopathy

PMR:

Polymyalgia rheumatica

PR3:

Proteinase 3

RPGN:

Rapidly progressive glomerulonephritis

SGS:

Subglottic stenosis

TA:

Temporal artery

TAK:

Takayasu’s arteritis

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Acknowledgments

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Authors and Affiliations

  1. Division of Rheumatology, Department of Medicine, Hospital for Special Surgery, New York, NY, USA

    Sebastian E. Sattui

  2. Scleroderma, Vasculitis and Myositis Center, Division of Rheumatology, Department of Medicine, Hospital for Special Surgery, Weill Cornell Medicine, New York, NY, USA

    Robert F. Spiera

Authors
  1. Sebastian E. Sattui
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  2. Robert F. Spiera
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Correspondence to Robert F. Spiera .

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Editors and Affiliations

  1. Mount Sinai Hospital, New York, NY, USA

    Yousaf Ali

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Sattui, S.E., Spiera, R.F. (2022). Vasculitis. In: Ali, Y. (eds) Rheumatology for Primary Care Providers. Springer, Cham. https://doi.org/10.1007/978-3-030-80699-6_14

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  • DOI: https://doi.org/10.1007/978-3-030-80699-6_14

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  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-030-80698-9

  • Online ISBN: 978-3-030-80699-6

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