• Churg-Strauss Syndrome (CSS), now also referred to by its medically more accurate term eosinophilic granulomatosis with polyangiitis (EGPA), is a rare systemic vasculitis (inflammation in the wall of blood vessels of the body), predominantly affecting small-sized vessels. (vasculitisfoundation.org)
  • Because antineutrophil cytoplasmic auto-antibodies (ANCA) can be detected in the serum (liquid and a cellular part of the blood) in up to 40% of the patients, it is also considered one of the three ANCA-associated vasculitides, along with granulomatosis with polyangiitis (Wegener's) and microscopic polyangiitis. (vasculitisfoundation.org)
  • Other possible secondary causes of cerebral vasculitis are infections, 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 other forms of systemic vasculitis such as granulomatosis with polyangiitis, polyarteritis nodosa or Behçet's disease. (wikipedia.org)
  • Granulomatosis with Polyangiitis (GPA) Granulomatosis with polyangiitis is characterized by necrotizing granulomatous inflammation, small- and medium-sized vessel vasculitis, and focal necrotizing glomerulonephritis, often with crescent. (merckmanuals.com)
  • Vasculitis is defined as inflammation of blood vessels. (medscape.com)
  • Primary" angiitis/vasculitis of the central nervous system (PACNS) is said to be present if there is no underlying cause. (wikipedia.org)
  • The exact mechanism of the primary disease is unknown, but the fundamental mechanism of all vasculitides is autoimmunity. (wikipedia.org)
  • This article focuses on the primary systemic vasculitides of childhood. (medscape.com)
  • Other subtypes of primary childhood vasculitis are much rarer. (medscape.com)
  • Practitioners may find it helpful to think of primary systemic vasculitides based on the predominant size of the involved vessels, as suggested by the Chapel Hill Classification. (medscape.com)
  • Primary vasculitis has no known cause. (merckmanuals.com)
  • The clinical spectrum of primary renal vasculitis. (medscape.com)
  • Vasculitis can also occur secondary to another disease, and this should be ruled out. (medscape.com)
  • Secondary vasculitis may be triggered by an infection, a drug, or a toxin or may occur as part of another inflammatory disorder or cancer. (merckmanuals.com)
  • Vasculitis is a family of rare diseases that can affect people of all ages. (vasculitisfoundation.org)
  • However, an overlap of vessel sizes affected within the diseases is noted, and the phenotype and pattern of organ involvement in vasculitis seems to be independent of vessel size. (medscape.com)
  • Central nervous system vasculitides are elusive diseases that are challenging to diagnose because brain biopsies have high false-negative rates. (ajnr.org)
  • See Vasculitis: Case Presentations , a Critical Images slideshow, for more information on clinical, histologic, and radiographic imaging findings in various forms of vasculitis. (medscape.com)
  • For example, Kawasaki disease is characterized by a mucocutaneous syndrome and coronary artery inflammation, whereas polyarteritis nodosa (PAN), another medium-vessel vasculitis, manifests with nodular skin lesions, neuropathy, and hypertension. (medscape.com)
  • the vessel inflammation (true vasculitis) is only part of the pathophysiology and there is predominant parenchymal inflammation in a characteristic pattern that involves specific organs. (merckmanuals.com)
  • As such, the classification of childhood vasculitides incorporates both vessel size and organ manifestations. (medscape.com)
  • As such, the most recent classification of childhood vasculitides incorporates both vessel size and organ manifestations. (medscape.com)
  • However, the clinical characteristics and outcomes of these three vasculitides differ noticeably. (vasculitisfoundation.org)
  • Characteristic clinical features are caused by stenotic large vessels and subsequently decreased blood supply to the organ systems. (medscape.com)
  • Cryogloblinemia is a vasculitis of small blood vessels that is caused by deposition of immune complexes: large aggregates of antibodies and the other proteins they are bound to. (vasculitisfoundation.org)
  • Some other types of vasculitis are also caused by immune complexes, but cryoglobulinemia is defined by a laboratory test that identifies immune complexes that fall out of solution in the cold. (vasculitisfoundation.org)
  • Henoch-Schönlein Purpura (HSP) is the most common vasculitis in children and is usually a benign disease that completely resolves in most patients without the need for aggressive treatment. (vasculitisfoundation.org)
  • The different types of vasculitis are classified according to the size and location of the blood vessels that are affected. (vasculitisfoundation.org)
  • If blood flow in a vessel with vasculitis is reduced or stopped, the parts of the body that receive blood from that vessel begins to die. (wikipedia.org)
  • Vasculitis can involve vessels of any size and can affect any organ system. (medscape.com)
  • Cerebral vasculitis is a very rare condition that is difficult to diagnose, and as a result there are significant variations in the way it is diagnosed and treated. (wikipedia.org)