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12 Cards in this Set
- Front
- Back
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name the Large vessel vasculitides
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o Giant cell arteritis (also temporal arteritis)
o Takayasu's arteritis |
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name the Medium-sized vessel vasculitides
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* Polyarteritis nodosa
* Kawasaki's disease * Cerebral vasculitis (primary granulomatous) |
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Name the Small-vessel vasculitis Associated with ANCAs (anti-neutrophil cytoplasmatic antibody):
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o Microscopic polyangiitis
o Wegener's granulomatosis o Churg-Strauss syndrome o Drug-induced |
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Name the small vessel vasculitides associated with deposition of immune complexes:
(11) |
* Henoch-Schönlein purpura (HSP)
* Cryoglobinemic vasculitis * Lupus erythematosus vasculitis * Rheumatoid vasculitis * Sjögren's syndrome vasculitis * Urticarial vasculitis associated with decreased complement * Behçet's disease * Goodpasture's syndrome * Serum sickness-vasculitis * Drug-induced * Infection-induced (not infectious) |
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Although the classification is not designed specifically as a diagnostic guideline, the presence of three of the following criteria point to the diagnosis of giant cell arteritis:
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Age 50 or over at onset
New onset of localized headache Temporal artery tenderness or decreased temporal artery pulse ESR of 50mm/h or more Abnormal temporal artery biopsy (showing mononuclear infiltration or granulomatous infiltration with giant cells) |
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biopsy results in temporal arteritis
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Panarteritis with predominantly lymphomononuclear infiltrate, giant cell granuloma formation, and disruption of the internal elastic lamina, intimal fibrosis, and thrombosis, often in short 'skip lesions'.
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Takayasu's arteritis Features
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Affects predominantly young asian women
Seldom starts after 40 years of age Vasculitis most commonly involves the aortic arch and its larger branches |
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The American College of Rheumatology, in it's 1990 criteria for the classification of polyarteritis nodosa, states that patients must have three of the following 10 criteria for a diagnosis of polyarteritis nodosa:
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Weight loss >4kg
Livedo reticularis Testicular pain or tenderness Myalgias, weakness, or leg tenderness Mononeuropathy or polyneuropathy Diastolic blood pressure >90mmHg Elevated blood urea nitrogen or creatinine Hepatitis B virus Arteriographic abnormality Biopsy of small or medium artery containing histologic evidence of vasculitis |
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How is polyarteritis nodosa differentiated from other vasculitis disorders?
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The key differences between polyarteritis nodosa and other necrotizing vasculitides are:
Lack of granuloma formation Sparing of veins, except by contiguous spread Sparing of pulmonary arteries Predilection for medium-sized arteries Absence of renal glomerular involvement |
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Microscopic polyangiitis is an ill-defined autoimmune disease which usually presents with pulmonary bleeding and anemia. Laboratory tests show:
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increased sedimentation rate,
reduced red blood count, antineutrophil cytoplasmic antibodies (ANCA) directed against myeloperoxidase (a constituent of neutrophil granules). protein and red blood cells in the urine. *The test for anti-glomerular basement membrane antibody (GBM), which is positive in Goodpasture's syndrome, is negative. |
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two types ANCA, pattern of staining on light microscopy, associated diseases:
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* pANCAs (perinuclear staining) - against myeloperoxidase - in Churg-Strauss syndrome, microscopic polyangiitis and various other diseases
* cANCAs (cytoplasmic staining) - against proteinase 3 - mainly in Wegener's granulomatosis |
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wegener's granulomatosis classic triad of features:
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necrotizing granulomatous inflammation of the upper and lower respiratory tracts; glomerulonephritis; and small-vessel vasculitis, involving the arteries and veins
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