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Miscellaneous Organ Involvement in ANCA-Associated Vasculitis

  • Giorgio Trivioli
  • Augusto Vaglio
Chapter
Part of the Rare Diseases of the Immune System book series (RDIS)

Abstract

ANCA-associated vasculitides (AAVs) are small-vessel vasculitides with a broad spectrum of manifestations, since almost all organs can be affected. Miscellaneous organ manifestations including the involvement of cardiovascular and musculoskeletal system, gastrointestinal and urogenital tract, occur in only a minority of patients but may have a profound impact on morbidity and mortality. As an example, cardiac involvement, including subclinical forms, is emerging as a diffuse and insidious disease component. In eosinophilic granulomatosis with polyangiitis (EGPA), cardiac abnormalities have been related to eosinophil infiltration and occur in up to 50% of patients, accounting for the first disease-related cause of death. Overall, AAV patients experience an increased frequency of ischemic heart disease, due to “accelerated atherosclerosis.” Gastrointestinal manifestations are not specific and may range from mild to life-threatening complications, even requiring surgery. A small number of granulomatosis with polyangiitis (GPA) male patients develop prostatitis as the most common urogenital manifestation, while therapy based on cyclophosphamide may cause hemorrhagic cystitis and urothelial cancer. In isolated cases, muscle vessels may be affected by vasculitis resulting in chronic ischemia and fiber degeneration. Although the list of manifestations is potentially infinite, it must be kept in mind that AAV may associate with a number of autoimmune and fibro-inflammatory diseases. Thus, patients with AAV should be carefully and periodically evaluated for new or modified manifestations and should receive a serological screening to exclude the development of overlap syndromes.

Keywords

ANCA-associated vasculitis Cardiovascular disease Endomyocarditis Venous thromboembolism Gastrointestinal disease Inflammatory bowel disease Cyclophosphamide Myositis IgG4-related disease 

Abbreviations

18FDG

18Fluorodeoxyglucose

AAV

ANCA-associated vasculitis

AKI

Acute kidney injury

ANA

Antinuclear antibody

ANCA

Antineutrophil cytoplasmic antibody

Anti-GBM

Anti-glomerular basal membrane

BD

Behcet’s disease

CD

Crohn’s disease

CK

Creatin kinase

CKD

Chronic kidney disease

CMR

Cardiac magnetic resonance

CT

Computed tomography

DVT

Deep venous thrombosis

ECG

Electrocardiography

EGPA

Eosinophilic granulomatosis with polyangiitis

EMB

Endomyocardial biopsy

EMG

Electromyography

ENT

Ear-nose-throat

EUVAS

European Vasculitis Study Group

FFS

Five-factor score

GI

Gastrointestinal

GPA

Granulomatosis with polyangiitis

HES

Hypereosinophilic syndrome

HLA

Human leukocyte antigen

IBD

Inflammatory bowel disease

IgAV

IgA vasculitis

IgG4-RD

IgG4-related disease

IHD

Ischemic heart disease

IMT

Intima-media thickening

LDH

Lactate dehydrogenase

LGE

Late gadolinium enhancement

MPA

Microscopic polyangiitis

MPO

Myeloperoxidase

MRI

Magnetic resonance imaging

NET

Neutrophil extracellular trap

PAN

Polyarteritis nodosa

PE

Pulmonary embolism

PET

Positron emission tomography

PNS

Peripheral nervous system

PR3

Proteinase 3

RA

Rheumatoid arthritis

RPF

Retroperitoneal fibrosis

RTX

Rituximab

SLE

Systemic lupus erythematosus

SOV

Single-organ vasculitis

SSc

Systemic sclerosis

STIR

Short-T1 inversion recovery

SVV

Small-vessel vasculitis

TF

Tissue factor

TNF

Tumor necrosis factor

UC

Ulcerative colitis

VTE

Venous thromboembolism

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Copyright information

© Springer Nature Switzerland AG 2020

Authors and Affiliations

  • Giorgio Trivioli
    • 1
  • Augusto Vaglio
    • 2
  1. 1.Department of Biomedical Experimental and Clinical Sciences “Mario Serio”University of FlorenceFlorenceItaly
  2. 2.Nephrology UnitMeyer’s Children HospitalFlorenceItaly

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