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GPA treatment depends on the severity of the disease. [8] Severe disease is typically treated with a combination of immunosuppressive medications such as rituximab or cyclophosphamide and high-dose corticosteroids to control the symptoms of the disease and azathioprine, methotrexate, or rituximab to keep the disease under control.
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.
Avacopan, sold under the brand name Tavneos, is a medication used to treat anti-neutrophil cytoplasmic autoantibody-associated vasculitis. [5] [6] [8] Avacopan is a complement 5a receptor antagonist [5] and a cytochrome P450 3A4 inhibitor.
The disease spectrum varies from failure of multiple organs to involvement of a single organ. Almost any organ could be impacted; however, polyarteritis nodosa rarely affects the lungs for unknown reasons. [10] Kawasaki disease (KD) is a type of systemic vasculitis of medium-sized vessels with an acute onset that primarily affects young children.
The clinician should ask about the duration, onset, and presence any associated symptoms such as weight loss or fatigue (that would indicate a systemic cause). [12] It is important to distinguish between IgA and non-IgA vasculitis. IgA vasculitis is more likely to present with abdominal pain, bloody urine, and joint pain. [13]
Eosinophilic granulomatosis with polyangiitis consists of three stages, but not all patients develop all three stages or progress from one stage to the next in the same order; [7] whereas some patients may develop severe or life-threatening complications such as gastrointestinal involvement and heart disease, some patients are only mildly affected, e.g. with skin lesions and nasal polyps. [8]
Possible secondary causes of cerebral vasculitis are infections such as with varicella zoster virus (chicken pox or shingles), systemic auto-immune diseases such as systemic lupus erythematosus (SLE) and rheumatoid arthritis, medications and drugs (amphetamine, cocaine and heroin), some forms of cancer (lymphomas, leukemia and lung cancer) and ...
While the mechanism of the disease has yet to be fully elucidated, the leading hypothesis is that AAV (ANCA Associated Vasculitis) develops in patients with a genetic predisposition when an unknown cause triggers the production of p-ANCA. These antibodies will circulate at low levels until an environmental trigger—such as infection ...
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