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Granulomatosis with polyangiitis is part of a larger group of vasculitic syndromes called systemic vasculitides or necrotizing vasculopathies, all of which feature an autoimmune attack by an abnormal type of circulating antibody termed ANCAs (antineutrophil cytoplasmic antibodies) against small and medium-size blood vessels.
Hughes–Stovin syndrome (HSS) is a rare autoimmune disorder often described as inflammation in relation to blood vessels, a form of vasculitis.It is not associated with any known cause and is typically characterized by multiple aneurysms in pulmonary arteries and deep vein thromboses.
Aortitis is most commonly seen in patients with syphilis, autoimmune vasculitis (giant cell arteritis, Takayasu's arteritis), polymyalgia rheumatica, and rheumatoid arthritis. [2] IgG4-related disease has more recently been identified as a cause of aortitis, and also as a cause of periaortitis (inflammation surrounding the aorta).
Necrotizing vasculitis, also called systemic necrotizing vasculitis, [1] is a general term for the inflammation of veins and arteries that develops into necrosis and narrows the vessels. [ 2 ] Tumors , medications, allergic reactions , and infectious organisms are some of the recognized triggers for these conditions, even though the precise ...
Takayasu's arteritis (TA), also known as aortic arch syndrome, nonspecific aortoarteritis, and pulseless disease, [2] is a form of large vessel granulomatous vasculitis [3] with massive intimal fibrosis and vascular narrowing, most commonly affecting young or middle-aged women of Asian descent, though anyone can be affected.
The signs and symptoms of microscopic polyangiitis may resemble those of granulomatosis with polyangiitis (GPA) (another form of small-vessel vasculitis) but typically lacks the significant upper respiratory tract involvement (e.g., sinusitis) frequently seen in people affected by GPA. [citation needed]
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]
Giant cell arteritis (GCA), also called temporal arteritis, is an inflammatory autoimmune disease of large blood vessels. [4] [7] Symptoms may include headache, pain over the temples, flu-like symptoms, double vision, and difficulty opening the mouth. [3]