Vasculitis Flashcards
What is Type 3 immune mechanism?
Immune complex deposition formed locally or deposited in areas of increased vascular permeability
What happens to complement and neutrophils in type 3?
Fixation of complement and infiltration of neutrophils
In severe cases what does type 3 cause?
Fibrinoid necrosis
Examples of type 3?
SLE
Serum sickness
Cryoglobulinemia
Chronic viruses
Cytoplasmic ANCA indicative of what?
Wegener’s granulomatosis
Perinuclear ANCA indicative of what?
Micro PAN
Are ANCA’s intrinsic part of pathogenesis or correlated phenomenon?
Unclear
What do ANCA’s serve as?
Diagnostic clinical marker
Granulomas associated with what?
Type 2 or 3 processes
Type 4 mechanisms present as?
Herpes and measles with no vasculitis
What infiltrate is there in type 2?
Leukocytes with direct cytotoxicity of endothelium
Type 3 infiltrates?
Immune complex deposition, complement fixation, and neutrophils
Type 4 infiltrate?
Monocytic infiltrate
What is associated with collections of eosinophils?
Allergic angiitis/Churg-Strauss syndrome
What are leukoclastic lesions?
Fragmentation of neutrophils
Where are granulomas in vascular wall?
Within vessel wall or directly adjacent to wall
What is fibrinoid necrosis?
Necrosis of cells within the vessel wall + accumulation of fibrin
Fibrinoid necrosis accompanies what?
Immune complex deposition and infiltration of leukocytes as in polyarteritis nodosa
How does hyalin accumulate?
Secondary to endothelial injury
Leakage of fibrin and synthesis of ECM
Long standing benign hypertension
What does hyper plastic lesions cause?
Activation of endothelial cells and proliferation of smooth muscle
Where are hyper plastic lesions seen?
Malignant hypertension, due to cytokines as in scleroderma
Chronic or repetitive damage to endothelium forms what?
Hyperplastic lesions
Polyarteritis Nodosa (Classic) presents where?
Small and medium arteries
PAN spares what organ?
Lungs
What do lesions of PAN look like?
Sharply segmental, branching points and bifurcations
In PAN what type of dilation occurs?
Segmental aneurysmal dilation, palpable nodule in skin
What causes the vessel wall weakening in PAN?
Inflammatory involvement
In PAN, renal involvement causes many deaths, how does this happen?
Due to renal arterioles, not glomerulonephritis
What do you see in acute phase of lesions?
Transmural infiltrates inflammatory cells and fibrinoid necrosis
Chronic phase of lesions?
Fibrous thickening of bessel wall/mononuclear infiltrate
Symptoms of PAN?
Melena (black tarry stools indicative of blood in feces)
Peripheral neuritis
Where does microscopic PAN occur?
Post capillary venules
What disorders are involved with Microscopic PAN?
Necrotizing glomerulonephritis (90%) and pulmonary capillary involvement.
In microscopic PAN what does cutaneous lesions manifest as?
Palpable purpura
What differs between microscopic PAN and normal PAN>
Lesions tend to be of the same age in microscopic
Characteristic of microscopic PAN?
Few or no immune deposits
P or C ANCA?
P (80%)
Giant cell arteritis occurs in who?
Older individuals
Where does Giant Cell Arteritis occur?
Temporal, vertebral, ophthalmic arteries. Rarely aortic arch
Symptoms of Giant Cell Arteritis?
Blindness, tenderness over involved arteries
What does Giant Cell Arteritis respond too?
Corticosteroids
Takayasu’s Arteritis occurs in who?
Women younger than 40
What does Takayasu’s Arteritis involve?
Origins of great arteries arising from aortic arch
Takayasu has what affect on the mediastinum?
Widening
How will Takayasu present?
Lower blood pressure and diminished or asymmetric pulses in upper extremities
Classical triad of Wegener’s Granulomatosis
Acute necrotizing granulomas
Granulomatous vasculitis
Renal disease with social/necrotizing glomerulonephritis
Wegener’s Granulomatosis associated with what?
Associated with epistaxis and hemoptysis and ulcerative lesions of nose, palate, pharynx
Good marker of disease activity in Wegener’s?
c-ANCA present in 90%
How quickly will Wegener’s kill you?
1 year
Kawasaki disease in who?
Young children
What type of lymph node syndrome is Kawasaki?
Mucocutaneous
