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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.
Vasculitis is a group of disorders that destroy blood vessels by inflammation. [2] Both arteries and veins are affected. Lymphangitis (inflammation of lymphatic vessels) is sometimes considered a type of vasculitis. [3] Vasculitis is primarily caused by leukocyte migration and resultant damage.
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.
Polyarteritis nodosa (PAN) is a systemic necrotizing inflammation of blood vessels (vasculitis) affecting medium-sized muscular arteries, typically involving the arteries of the kidneys and other internal organs but generally sparing the lungs' circulation. [3]
In the setting of systemic vasculitis as described above, proliferative nephritis is associated with antineutrophil cytoplasmic antibodies (ANCA). [3] Because of this, an ANCA test should always follow a negative immunofluorescence result to have the highest accuracy for confirming pauci-immune vasculitis-driven proliferative nephritis. [1]
Vasculitic neuropathy is a peripheral neuropathic disease. [1] [2] [3] In a vasculitic neuropathy there is damage to the vessels that supply blood to the nerves.[1] [2] [3] It can be as part of a systemic problem or can exist as a single-organ issue only affecting the peripheral nervous system (PNS).
There are some concerns with the use of anti-TNF agents in patients with systemic autoimmune diseases due to the risk of triggering disease exacerbations. [ 2 ] The term polyangiitis overlap syndrome refers to a systemic vasculitis that shares features with two or more distinct vasculitis syndromes.
Retinal vasculopathy with cerebral leukocencephalopathy and systemic manifestations (RVCL or RVCL-S, also previously known as retinal vasculopathy with cerebral leukodystrophy, RVCL; or cerebroretinal vasculopathy, CRV; or hereditary vascular retinopathy, HVR; or hereditary endotheliopathy, retinopathy, nephropathy, and stroke, HERNS) is an inherited condition resulting from a frameshift ...