ANCA-associated vasculitides
Encyclopedia
ANCA-associated vasculitides (AAV) are diseases caused by vasculitis
in which antineutrophil cytoplasmic antibodies (ANCAs) can be detected in the blood.
, microscopic polyangiitis
and Wegener's granulomatosis
. All feature a small-vessel vasculitis.
Polyarteritis and pauci-immune
rapidly progressive glomerulonephritis
also feature ANCA antibodies. Some cases of polyarteritis nodosa
, especially presenting microscopic polyangiitis
, will be p-ANCA
positive.
Vasculitis
Vasculitis refers to a heterogeneous group of disorders that are characterized by inflammatory destruction of blood vessels. Both arteries and veins are affected. Lymphangitis is sometimes considered a type of vasculitis...
in which antineutrophil cytoplasmic antibodies (ANCAs) can be detected in the blood.
Types
Three main AAVs are Churg-Strauss syndromeChurg-Strauss syndrome
Churg–Strauss syndrome is a medium and small vessel autoimmune vasculitis, leading to necrosis. It involves mainly the blood vessels of the lungs , gastrointestinal system, and peripheral nerves, but also affects the heart, skin and kidneys. It is a rare disease that is non-inheritable and...
, microscopic polyangiitis
Microscopic polyangiitis
Microscopic polyangiitis is an ill-defined autoimmune disease characterized by pauci-immune, necrotizing, small-vessel vasculitis without clinical or pathological evidence of necrotizing granulomatous inflammation.-Presentation:Because many different organ systems may be involved, a wide range of...
and Wegener's granulomatosis
Wegener's granulomatosis
Wegener's granulomatosis , more recently granulomatosis with polyangiitis , is an incurable form of vasculitis that affects the nose, lungs, kidneys and other organs. Due to its end-organ damage, it is life-threatening and requires long-term immunosuppression...
. All feature a small-vessel vasculitis.
Polyarteritis and pauci-immune
Pauci-immune
Pauci-immune is a general term used to refer to a form of vasculitis that is associated with minimal evidence of hypersensitivity upon immunofluorescence.They can be associated with antineutrophil cytoplasmic antibodies ....
rapidly progressive glomerulonephritis
Rapidly progressive glomerulonephritis
Rapidly progressive glomerulonephritis is a syndrome of the kidney that is characterized by a rapid loss of renal function, with glomerular crescent formation seen in at least 50% or 75% of glomeruli seen on kidney biopsies...
also feature ANCA antibodies. Some cases of polyarteritis nodosa
Polyarteritis nodosa
Polyarteritis nodosa is a vasculitis of medium & small-sized arteries, which become swollen and damaged from attack by rogue immune cells. Polyarteritis nodosa is also called Kussmaul disease or Kussmaul-Maier disease...
, especially presenting microscopic polyangiitis
Microscopic polyangiitis
Microscopic polyangiitis is an ill-defined autoimmune disease characterized by pauci-immune, necrotizing, small-vessel vasculitis without clinical or pathological evidence of necrotizing granulomatous inflammation.-Presentation:Because many different organ systems may be involved, a wide range of...
, will be p-ANCA
P-ANCA
p-ANCA, or Perinuclear Anti-Neutrophil Cytoplasmic Antibodies, show a perinuclear staining pattern.This pattern occurs because the vast majority of the antigens targeted by ANCAs are highly cationic at pH 7.00. During ethanol fixation, antigens which are more cationic migrate and localize around...
positive.