Our understanding of AAV is increasing

AAV (ANCA-associated vasculitis) is a group of progressive, rare, and severe autoimmune diseases.^1,2

AAV can affect blood vessels in different parts of the body resulting in damage to vital organs such as the lungs, kidneys, nervous system, gastrointestinal system, skin, eyes, and heart.²

The three main clinical subtypes of AAV are GPA (granulomatosis with polyangiitis, previously called Wegener’s), MPA (microscopic polyangiitis) and EGPA (eosinophilic granulomatosis with polyangiitis, previously called Churg-Strauss syndrome).^3,4 Both GPA and MPA show a lot of clinical commonality, whereas EGPA is distinctly different.⁵ For this reason, this website will focus mainly on GPA and MPA.

References & footnotes

AAV, ANCA-associated vasculitis; ANCA, anti-neutrophil cytoplasmic antibody; EGPA, eosinophilic granulomatosis with polyangiitis; GPA, granulomatosis with polyangiitis; MPA, microscopic polyangiitis

1. Hutton HL, et al. Semin Nephrol 2017;37(5):418–35.
2. Al-Hussain T, et al. Adv Anat Pathol 2017;24(4):226–34.
3. Jennette JC, et al. Arthritis Rheum 2013;65(1):1–11.
4. Yates M, Watts R. Clin Med (Lond) 2017;17(1):60–4.
5. Pagnoux C. Eur J Rheumatol 2016;3(3):122–33.