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12 Cards in this Set

  • Front
  • Back
name the Large vessel vasculitides
o Giant cell arteritis (also temporal arteritis)
o Takayasu's arteritis
name the Medium-sized vessel vasculitides
* Polyarteritis nodosa
* Kawasaki's disease
* Cerebral vasculitis (primary granulomatous)
Name the Small-vessel vasculitis Associated with ANCAs (anti-neutrophil cytoplasmatic antibody):
o Microscopic polyangiitis
o Wegener's granulomatosis
o Churg-Strauss syndrome
o Drug-induced
Name the small vessel vasculitides associated with deposition of immune complexes:
* 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)
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:
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)
biopsy results in temporal arteritis
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'.
Takayasu's arteritis Features
Affects predominantly young asian women
Seldom starts after 40 years of age
Vasculitis most commonly involves the aortic arch and its larger branches
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:
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
How is polyarteritis nodosa differentiated from other vasculitis disorders?
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
Microscopic polyangiitis is an ill-defined autoimmune disease which usually presents with pulmonary bleeding and anemia. Laboratory tests show:
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.
two types ANCA, pattern of staining on light microscopy, associated diseases:
* 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
wegener's granulomatosis classic triad of features:
necrotizing granulomatous inflammation of the upper and lower respiratory tracts; glomerulonephritis; and small-vessel vasculitis, involving the arteries and veins