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It is an autoimmune disease and a form of vasculitis that affects small- and medium-sized vessels in many organs, but most commonly affects the upper respiratory tract, lungs, and kidneys. [6] The signs and symptoms of GPA are highly varied and reflect which organs are supplied by the affected blood vessels.
The most frequent presenting signs and symptoms of primary CNS vasculitis were focal neurological deficits (seen in 63% of cases), headaches (51%) and cognitive impairment (41%). [4] Other presenting symptoms include aphasia or other difficulties with speech (35-43%), ataxia , visual field deficits, acute or subacute encephalopathy (which may ...
c-ANCA/PR3-ANCA Confirmed [36] IgA vasculitis (IgAV) Small vessels in skin, joints, kidneys, gastrointestinal tract IgA immune complexes Probable [37] Leukocytoclastic vasculitis: Small vessels in skin Various immune complexes Probable [38] Lupus vasculitis: Small to medium vessels in multiple organs ANA, anti-dsDNA, anti-Smith, others ...
The definite diagnosis of vasculitis is established after a biopsy of involved organ or tissue, such as skin, sinuses, lung, nerve, brain, and kidney. The biopsy elucidates the pattern of blood vessel inflammation. Some types of vasculitis display leukocytoclasis, which is vascular damage caused by nuclear debris from infiltrating neutrophils. [37]
Atypical ANCA is associated with drug-induced systemic vasculitis, inflammatory bowel disease and rheumatoid arthritis. [3] [7] The ANCA-positive rate is much higher in patients with type 1 diabetes mellitus than in healthy individuals. [8] Levamisole, which is a common adulterant of cocaine, can cause an ANCA positive vasculitis. [9]
Rapidly progressive glomerulonephritis (RPGN) is a syndrome of the kidney that is characterized by a rapid loss of kidney function, [4] [5] (usually a 50% decline in the glomerular filtration rate (GFR) within 3 months) [5] with glomerular crescent formation seen in at least 50% [5] or 75% [4] of glomeruli seen on kidney biopsies.
ANCA will less commonly form against alternative antigens that may also result in a p-ANCA pattern. These include lactoferrin, elastase, and cathepsin G. [citation needed] When the condition is a vasculitis, the target is usually MPO. [1] However, the proportion of p-ANCA sera with anti-MPO antibodies has been reported to be as low as 12%. [2]
The disease's symptoms can range from catastrophic neurological impairment to an asymptomatic condition brought on by impalpable pulses or bruits. [7] Non-specific features include mild anemia, myalgia, arthralgia, weight loss, malaise, night sweats, and fever. [8] Giant cell arteritis (GCA) is the most common type of systemic vasculitis in adults.