Large vessel<unk>  Giant-cell arteritis and Takayasu's arteritis have much in common, but usually affect patients of different ages, with Takayasu's arteritis affecting younger people, and giant-cell arteritis having a later age of onset. Aortitis can also be considered a large-vessel disease. Takayasu arteritis. Primarily affects the aorta and its main branches. At least three out of six criteria yields sensitivity and specificity of 90.5 and 97.8%: * Onset 4 kg * Livedo reticularis * Testicular pain * Myalgias, weakness * Abdominal pain, diarrhea, and GI bleeding * Mononeuropathy or polyneuropathy * New onset diastolic blood pressure > 90 mmHg * Elevated serum blood urea nitrogen (> 40 mg/dL) or serum creatinine (> 1.5 mg/dL) * Hepatitis B infection * Arteriographic abnormalities * Arterial biopsy showing polymorphonuclear cells Kawasaki disease. Usually in children (age 1.5 cm) Isolated cerebral<unk> . Affects medium and small arteries over a diffuse CNS area, without symptomatic extracranial vessel involvement. Patients have CNS symptoms as well as cerebral<unk>  by angiography and leptomeningeal biopsy. Small vessel<unk>  There are several<unk> es that affect small vessels. Pauci-immune Granulomatosis with polyangiitis.<unk>  of medium and small arteries, including venules and arterioles. Produces granulomatous inflammation of the respiratory tracts and necrotizing, pauci-immune glomerulonephritis. Most common cause of saddle nose deformity in USA (nose flattened due to destruction of nasal septum by granulomatous inflammation). Almost all patients with granulomatosis with polyangiitis have c-antineutrophil cytoplasmic antibody, but not vice versa. Current treatment of choice is cyclophosphamide. At least two out of four criteria yields sensitivity and specificity of 88 and 92%. * Nasal or oral inflammation (oral ulcers or purulent/bloody nasal discharge, may be painful) * Abnormal chest X-ray with showing nodules, infiltrates, or cavities * Microscopic hematuria or red blood cell casts * Vessel biopsy shows granulomatous inflammation *Peak incidence: ages 40–60, males > females Eosinophilic granulomatosis with polyangiitis (formerly known as Churg–Strauss syndrome). Affects medium and small vessels with vascular and extravascular granulomatosis. Classically involves arteries of lungs and skin, but may be generalized. At least four criteria yields sensitivity and specificity of 85 and 99.7%. * Asthma (history of wheezing or presently wheezing) * Eosinophilia > 10% on complete blood count * Mononeuropathy or polyneuropathy * Migratory or transient pulmonary opacities on chest x-ray * Paranasal sinus abnormalities * Vessel biopsy showing eosinophils in extravascular areas Microscopic polyarteritis/polyangiitis. Affects capillaries, venules, or arterioles. Thought to be part of a group that includes granulomatosis with polyangiitis since both are associated with antineutrophil cytoplasmic antibody and similar extrapulmonary manifestations. Patients do not usually have symptomatic or histologic respiratory involvement. Immune complex Hypersensitivity<unk>  (allergic<unk> . Usually due to a hypersensitivity reaction to a known drug. Drugs most commonly implicated are penicillin, sulphonamides and thiazide diuretics. Methamphetamine and other sympathomimetics can cause a cerebral<unk>  alongside polyarteritis nodosa like<unk>  features. With other drugs,there is presence of skin<unk>  with palpable petechiae or purpura. Biopsy of these lesions reveal inflammation of the small vessels, termed leukocytoclastic<unk> , which is most prominent in postcapillary venules. At least three out of five criteria yields sensitivity and specificity of 71 and 84%: * Age > 16 * Use of possible triggering drug in relation to symptoms * Palpable purpura * Maculopapular rash * Skin biopsy showing neutrophils around vessel IgA<unk>  (formerly known as Henoch–Schonlein purpura).<unk>  due to tissue deposition of IgA-containing immune complexes. Biopsy of lesions shows inflammation of small vessels. It is considered a form of hypersensitivity<unk>  but is distinguished by prominent deposits of IgA. This is the most common<unk>  in children. Presence of three or more criteria yielded sensitivity of 87% while less than two criteria yielded hypersensitivity<unk>  in 74%: * Palpable purpura (usually of buttocks and legs) * Bowel angina * GI bleed * Hematuria * Onset < 20 years * No new medications Essential cryoglobulinemic<unk> . Most often due to hepatitis C infection, immune complexes of cryoglobulins – proteins that consists of immunoglobulins and complement and precipitate in the cold while dissolving upon rewarming – are deposited in walls of capillaries, venules, or arterioles. Therefore, complement will be low with histology showing vessel inflammation with immune deposits. Other/ungrouped<unk>  secondary to connective tissue disorders. Usually secondary to<unk>  lupus erythematosus (SLE), rheumatoid arthritis (RA), relapsing polychondritis, Behçet's disease, and other connective tissue disorders.<unk>  secondary to viral infection. Usually due to hepatitis B and C, human immunodeficiency virus, cytomegalovirus, Epstein–Barr virus, and parvovirus B19.
Systemic vasculitis
Lupus vasculitis
Rheumatoid vasculitis
Livedoid vasculitis
Urticarial vasculitis
Bullous small vessel vasculitis
Systemic vasculitides
IgA vasculitis