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Miscellaneous Organ Involvement in ANCA-Associated Vasculitis

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Anti-Neutrophil Cytoplasmic Antibody (ANCA) Associated Vasculitis

Part of the book series: Rare Diseases of the Immune System ((RDIS))

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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.

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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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Authors and Affiliations

  1. Department of Biomedical Experimental and Clinical Sciences “Mario Serio”, University of Florence, Florence, Italy

    Giorgio Trivioli

  2. Nephrology Unit, Meyer’s Children Hospital, Florence, Italy

    Augusto Vaglio

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  1. Giorgio Trivioli
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  2. Augusto Vaglio
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  1. Department of Medicine and Surgery, University of Milano-Bicocca, Monza, Monza e Brianza, Italy

    Renato Alberto Sinico

  2. Department of Medicine, Paris Descartes University Hospital Cochin, Paris, France

    Loïc Guillevin

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Trivioli, G., Vaglio, A. (2020). Miscellaneous Organ Involvement in ANCA-Associated Vasculitis. In: Sinico, R., Guillevin, L. (eds) Anti-Neutrophil Cytoplasmic Antibody (ANCA) Associated Vasculitis. Rare Diseases of the Immune System. Springer, Cham. https://doi.org/10.1007/978-3-030-02239-6_15

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