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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.
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.
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.
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 ...
The most common treatment for DADA2 is TNF inhibitors. This therapy tends to prevent vasculitis-related manifestations such as rash and stroke, [5] [6] but does not perform well in individuals presenting with severe hematologic and immunologic abnormalities such as bone marrow failure or severe recurrent infections. [7]
In cases where a cause can be determined, medications and infectious pathogens are most common in adults, while IgA vasculitis (Henoch–Schönlein purpura) frequently affects children. [6] Other etiologies include autoimmune conditions and malignancies, usually hematologic (related to the blood).
Rituximab, sold under the brand name Rituxan among others, is a monoclonal antibody medication used to treat certain autoimmune diseases and types of cancer. [18] It is used for non-Hodgkin lymphoma, chronic lymphocytic leukemia (in children and adults, but not recommended in elderly patients), rheumatoid arthritis, granulomatosis with polyangiitis, idiopathic thrombocytopenic purpura ...
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