Vasculitis Flashcards
How do immune processes leading to vasculitis progress in the large vessel?
outside-in
- Activation of naive t-cells by tissue resident DCs in adventitia
- oxidative damage
- Non-stenosing arteritis (window of opportunity)
- hypertrophy of intima and lumen occlusion. symptoms occur. scar is forming
- On top of chronic stenosis you can get a thrombosis and the intima gets thicker–>occlusion
- breakdown of internal elastic lamina–>aneurysm formation
How do immune processes leading to vasculitis progress in the small/medium vessel?
inside out
a. Immune complexes
b. Antineutrophil antibodies
c. direct endothelial adhesion
- primary neutrophil
- neutrophil goes in and degranulates and causes inflammation
small–> breaking open of the artery
What are common features of all vasculitides?
- fatigue
- fever
- muscle pain
- weight loss
- *CRP
- *Fibrinogen (sedimentation rate)
What is the pathophysiology of small vessels?
- Fibrinoid Necrosis
- fibrin deposition –>breaking open the blood vessel
- dead neutrophils fibrin deposition–>fibrin necrosis
What are typical findings in small vessel vasculitis?
- palpable purpura
- necrotizing glomerulonephritis
- pulmonary capillaritis and alveolar hemorrhage
What is the pathophysiology of medium vessels?
- focal lesions with fibrinoid necrosis
- segmental lesions
- aneurysms
What are typical clinical findings of medium vessel vasculitis?
- skin nodules
- livedo racemosa
- ulceration
- nerve infarcts
- abdominal pain
What is the pathophysiology of large vessels?
- cell mediated
- not neutrophil or immune complex - granulomas usually present
- Hyperplastic intima with stenosis
- with neovascularization - FIBRINOID NECROSIS UNUSUAL
What are typical clinical findings of large vessel vasculitis?
- ocular symptoms
- Claudication
- chest pain
- headache stroke
What type of study do you use to look at large and medium arteries?
Angiography or MRA
What type of study do you use to look at small and medium arteries?
- CT chest
- lung/kidney biopsy
- skin biopsy
C-ANCA
PR3
P-ANCA
MPO
What are 4 clinical diagnosis considerations?
1) vessel size
2) anca association
3) presence of granulomas
4) possible disease association
What vasculitides have granulomas?
- Giant cell arteritis
- takayasu arteritis
- wegener granulomatosis
- churg strauss
Giant Cell Arteritis
Epidemiology
Vessel
Epidemiology:
1. greater than 50, white, female
- a. Carotid
i. External
- especially temporal arteries
ii. Internal
- ophthalmic
- not intracerebral
b. Upper aortic branches
i. vertebral arteries
NO STRoKES
What are the pathological features of GCA?
- patchy/segmental
- granulomatous inflammation
- focused on the internal elastic membrane - fragmentation of internal elastic membrane
chronic-may be smouldering even if clinically quiet
What are clinical features of GCA?
- headaches
- polymyalgia rheumatica
- scalp tenderness **
- TA (temporal artery) pulse abnormality **
- ocular disturbances
- visual loss
- extreme claudication
- cardiac or neurologic symptoms
How do you investigate for GCA?
temporal artery biopsy**
blood vessels imagin
What is the treatment for GCA?
corticosteroids with slow taper
What is the epidemiology and artery for pulseless disease (takayasu’s arteritis)?
50 or less
asian female
Aorta Great
Great vessels
STROKES
-favors bifurcation areas
What are the pathologic features of takayasu’s?
- giant cells (more spread)
- transmural scarring more intense
- severe hyperplastic intima with neovascularization
- severe luminal narrowing
What are the three phases of takayasu’s arteritis?
- pre-pulseless, inflammation period. patients under 20 usually present in this phase
- vessel inflammation dominated by vessel pain and tenderness
- fibrotic stage, when bruits and ischemia predominate
- arterial bypass or angioplasty for things like heart failure and htn often needed*
What are the clinical features of takayasu’s?
- abnormal pulses
- claudication/weakness
- pulmonary hypertension
- carotidynia
What is fundamental to diagnosis of takayasu’s?
Blood vessel imaging**
Echocardiography
- non specific lab tests
- ESR can be normal in 50%
What is the epidemiology of kawasaki’s?
children
What are the pathological features of kawaskis?
self limiting
-anti-endothelial/smooth muscle cell antibodies
What is window of opportunity to treat in kawasaki?
Stage 1: 0-2 weeks after fever
-perivasculitis of microvessels
Other stages
Stage 2: 2-4 weeks
-progression to medium vessel vasculitis
-thrombosis and aneurysms
Stage 3:
reduction in inflammation
Stage 4: scar with weakened wall and intimal thickening
What are clinical features of kawasaki’s?
fever rash bilateral conjunctivitis, strawberry tongue, extremity changes, cervical lymphadenopathy, MI
What do you do to treat kawasaki’s?
IVIG
High dose aspirin
within 1st 10 days of fever
incidence of coronary aneurysm 25–>3-5%
What is the epidemiology of PAN?
medium vessel
uncommon, all ages
associated-
autoimmune
heme malignancies
viruses- HEP B 30% *****
What vessels are affected by PAN?
medium to small MUSCULAR arteries
- renal
- coronary
- hepatic
What are the pathologic features of PAN?
- focal *
- segmental *
- transmural destruction *
- coexisting stages of activity*
- fibrinoid necrosis
- pleomorphic cellular infiltrate
- thrombosis
- aneurysms
What are clinical features of PAN?
- organ infarcts *
- abdominal injury
- renal injury/ hypertension
- cutaneous lesions
- arthralgia/arthritis
- acute peripheral neuropathy
- pain/paresthesia
- motor deficits hours later
- mononeuritis multiplex
Does PAN need treatment? What is the treatment?
rapidly progressive and fatal if not treated
Treatment
-corticosteroids
-HBV associated-plasma exchange, antiviral treatment
-cyclophosphamide chemotherapy if severe
Out of GPA (granulomatosis with polyangiitis-Wegners) , MPA (microscopic polyangiitis) and CS (churg-strauss) which are associated with granulomas?
Granulomatosis with polyangiitis
Churg strauss
What clinical feature of granulomatosis with polyangiitis?
- Destructive upper airway disease
- pulmonary nodules/cavities/infiltrates
- Necrotizing crescentic glomerulonephritis
- destructive sinusitis
- subglottis stenosis
- gingival hyperplasia
- orbital pseudotumor
- necrotizing scleritis
C-ANCA/PR3
What clinical feature of microscopic polyangiitis?
Alveolar hemorrhage
-Necrotizing crescentic glomerulonephritis
P-ANCA/MPO
What clinical feature of churg strauss?
Nasal polyps/allergic rhitnitis
Asthma, allergy, eosinophilia
-Necrotizing crescentic glomerulonephritis
-cardiomyopathy due to eosinophilic cardiac infiltrate
P-ANCA/MPO
What do you see in the urine of these three small vessel things?
proteinuria
RBC casts +/-
What is epidemiology of HSP?
most frequently children
-can occur in adults
small vessels
-arterioles, capillary, or venules
What are the pathologic features of HSP?
immune complex deposition
IgA and C3
What are the clinical features of HSP?
palpable purpura
arthritis
abdominal pain
renal impairment
Buerger?
smoking disease
medium and small arteries
gangrene of extremities
corkscrew collaterals
Which diseases can be treated with steroids?
- GCA
- Takayasu’s
- HSP
