Miscellaneous Organ Involvement in ANCA-Associated Vasculitis

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


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.


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





ANCA-associated vasculitis


Acute kidney injury


Antinuclear antibody


Antineutrophil cytoplasmic antibody


Anti-glomerular basal membrane


Behcet’s disease


Crohn’s disease


Creatin kinase


Chronic kidney disease


Cardiac magnetic resonance


Computed tomography


Deep venous thrombosis




Eosinophilic granulomatosis with polyangiitis


Endomyocardial biopsy






European Vasculitis Study Group


Five-factor score




Granulomatosis with polyangiitis


Hypereosinophilic syndrome


Human leukocyte antigen


Inflammatory bowel disease


IgA vasculitis


IgG4-related disease


Ischemic heart disease


Intima-media thickening


Lactate dehydrogenase


Late gadolinium enhancement


Microscopic polyangiitis




Magnetic resonance imaging


Neutrophil extracellular trap


Polyarteritis nodosa


Pulmonary embolism


Positron emission tomography


Peripheral nervous system


Proteinase 3


Rheumatoid arthritis


Retroperitoneal fibrosis




Systemic lupus erythematosus


Single-organ vasculitis


Systemic sclerosis


Short-T1 inversion recovery


Small-vessel vasculitis


Tissue factor


Tumor necrosis factor


Ulcerative colitis


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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