Vasculitis: big picture. What is the definition?
Inflammatory destruction of blood vessels. Many different causes.
Vasculitis: what can it cause? (3)
-ischemia, embolism, aneurysm
vasculitis: typically in veins or arteries?
can be either but more frequently affects arteries
This image depicts what? What type of vasculitis?
polyarteritis nodosum: example of vasculitis affecting medium sized arteries (coronary arteries) -> lumpy-bumpy arteries.
Histology of vasculitis. What is notable?
- narrowed lumen
- damaged epithelium (can lead to clots)
- many neutrophils due to inflammatory process. May also have eosinophils, lymphocytes.
How can we tell this is vasculitis?
Is this vasculitis of small, med or large vessels?
Is there a specific name for this?
-often on dependent areas (due to hydrostatic pressure)
This is of SMALL vessels
Henoch-Schoenlein Purpura presentation? (4)
(Small vessel vasculitis)
- palpable purpura
- associated with bloody diarrhea due to vasculitis of the bowel lining
Henoch-Schoenlein Purpura: what will be seen on biopsy?
neutrophilic leukocytic process with IgA present in vessels.
This destroys the vessels, WBCs leak out.
Henoch-Schoenlein Purpura: what is the cause?
When does it usually occur?
What causes a similar-looking rash?
HS Purpura is idiopathic
Occurs post-URI infection (vasculitis due to IgA complex deposition)
Similar: vasculitis due to drug reactions; connective tissue diseases
besides stellate ulcers/palpable purpura, what are skin clues that indicate small vessel vasculitis? (3)
livido reticularis (reticulated purpura)
What vasculitis is this picture characteristic of?
due to small vessel vasculitis
What does this picture show? due to what type of vasculitis?
stellate ulcers and gangrene
due to small vessel vasculitis
What does this pic show? due to what type of vascuiltis?
(net like pattern, usually more violet than red)
Usually on legs, arms, flanks
due to small vessel vasculitis
Henoch-Schoenlen Purpura. What is the typical clinical presentation?
Small vessel vasculitis.
Usually presents as
- palpable purpura
- abdominal pain
- GI bleeding
- with fever and malaise in a child.
What is this, what is it due to?
stellate lesion due to small vessel vasculitis
What is this, what type of vasculitis is it due to?
same situation as the heart image: due to aneurysmal dilation (in the vascular arch of the hand this time).
Polyarteritis nodosa: what size vessels are involved? what happens to those vessels?
Medium vessel vasculitis, vessels develop spasms + aneurysms.
Associated sx: gut ischemia, coronary aneurysms, renal artery involvement
Polyarteritis nodosa: usually secondary to what?
also seen in RA, Lupus, Kawasaki's disease (kids)
Polyarteritis nodosa: what are the immune cells that are primarily involved?
What is this? What size vessels are involved?
Heart with polyarteritis nodosa
What is depicted here?
note the vessel wall at the top of the pic is damaged (even though the rest looks normal) and it forms an aneurysm with inflamation surrounding it -> Polyarteritis Nodosum.
What is this?
Classic polyarteritis nodosum as seen via angiography
The aneurysms are 'beads'
What about the physical exam or symptoms should cause consideration of vasculitis? (5)
Skin lesions: palpable purpura, stellate lesions, punched out ulcers
Glomerulonephritis (may be due to vasculitis)
Mononeuritis multiplex (nerve infarction, may result in foot drop, hand drop)
Diseases of the elderly presenting in the young (signs of artery dz in a pt too young for atherosclerosis)
Systemic symptoms (B symptoms) in the absence of infection or malignancy
Primary vasculitis: what is the etiology? A few examples?
Primary: etiology is unknown.
Examples: Henoch-Schoenlen Purpura, Wegener's, Kawasaki's, Giant Cell Arteritis
(for a lot of the weird-named ones we don't know the cause)
Secondary vasculitis: what is the etiology?
May be associated with another connective tissue disease (lupus, RA)
may be infection related (Hep B/C, Parvo, Herpes, bacterial endocarditis)
Cryoglobulins (cold-precipitating proteins)
Primary vasculitis: even though we don't know the precipitating agent, the process is understood. What is the pathophysiology?
immune complex deposition (or in situ formation) in the vascular wall.
Leads to inflammation, cytokines, general immune response.
Location of depositions due to adhesion molecules in vascular beds, also turbulence patterns in blood flow, hydrostatic presure.
Likely there is a genetic predisposition that is triggered by environment or infection.
What is the classic presentation of Wegener's Granulomatosis? (now called Granulamotosis with Polyangitis)?
35 yo man, eye inflammation, sinusitis, lung lesions, hemoproteinuria and gangrene on fingers
What is seen here?
Wegener's/Granulamotosis with Polyantitis (GPA)
Left: extravascular granulamatous inflammation
Triad of symptoms for Granulomatosis with Polyangiitis (GPA)?
- Extravascular granuloma formation in upper or lower resp tract
- Pauci-immune glomerulonephritis
In general, lab tests in vasculitis are helpful how?
Usually they are nonspecific, and help more in defining the extent of the process than the disease itself.
pANCA and cANCA are exceptions to this generality.
What is ANCA?
What are the two different types?
Anti-Neutrophil Cytoplasmic Antibodies
Test for antibodies directed at enzymes and proteins within granules in neutrophils.
2 types: pANCA, cANCA
cANCA: what is the antigen?
serine proteinase 3 (PR3)
pANCA: what is the antigen?
What is shown on the left side? right side? of this pic
Left: cANCA (cytoplasmic staining pattern)
Right: pANCA (perinuclear staining pattern)
cANCA and pANCA: how should they be interpreted?
cANCA = Wegener's
pANCA = Churg-Strauss & microscopic polyarteritis
May be positive with other vasculitides, infections, etc.
Lots of false positives
--> sensitive but not specific
What are the ANCA-positive vasculities? (3)
What is their common presentation?
ANCA-positive vasculitides are
Wegener's and Microscopic Polyarteritis have a characteristic pulmonary-renal syndrome.
All 3 have lung involvement
she didn't talk about Microscopic Polyarteritis, but it presents like Wegener's (skin, kidneys, lungs), with diffuse alveolar hemorrhage. pANCA (specificity for MPO) will be positive.
Giant Cell Arteritis: what is classic presentation?
70 yo man, headaches where his hat rubs on his temples. scalp is painful, visual loss (falling curtain pattern), jaw hurts with chewing. Wt loss of 10# and low grade fever.
What does this depict?
Should you be more alarmed if you HAVE a pulse in the prominent artery, or if you do NOT?
Giant Cell Arteritis in the superficial temporal artery
If it is pulsatile, it is likely not GCA.
GCA: what are the qualities of the prominent arteries?
They will be tender, firm, and non-pulsatile (due to inflammation -> occlusion)
GCA: vasculitis of what size of vessels?
large vessel arteritis
GCA: what part of the artery is being destroyed? by what kind of cell?
The elastic lamina of muscular arteries is being destroyed, by monocytes.
Disease that is related to GCA but more systemic? Prevalent in what populations?
Takayasus aortitis. (large vessel vasculitis)
Sx = claudication of extremeties, hypertension, vision changes, dizziness, imbalance.
Prev in Asians and women.
Sucks for you, Jullet.
What is depicted?
Giant Cell Arteritis of temporal artery.
the internal elastic lamina is ok on the left side (looks like ribbon candy) but is destroyed on the right
see some giant cells (she says)
what is the best way to make a diagnosis of vasculitis?
Considerations for a biopsy to diagnose vasculitis?
-choose tissue that is symptomatic
-skin, muscle, nerve are more accessible than kidney, lung
-bigger chunk = better. lesions may be intermittent. for temporal artery, want 4 cm
-new lesions better than old
Some diseases present like vasculitis, but are not. What are a few of them?
Vasculopathies: processes that cause vessel obstruction that is non-inflammatory.
Cholesterol emboli, subacute bacterial endocarditis, DIC, amyloid, etc etc
Treatment for vasculitis?
-Remove the problem (if known)
-treat the inflammation with steroids (PREDNISONE) or steroid-sparing immunosuppressives
-treat damaged skin, hypertension
-monitor for further damage
Small vessel vasculitis (4)
Henoch Schoenlen Purpura
Granulomatosis w. Polyangiitis
Medium vessel vasculitis? (2)
Large vessel vasculitis? (2)
Giant cell Artertitis (aka Temporal arteritis)
vasculitis that forms granulamtous inflammation (4)
vasculitis that forms fibrinoid necrosis (1)
- Granulomatosis w. Polyangiitis
- temporal Arteritis
- Takaysku Arotitis
- Polyarteritis nodosa
What type of vasculitis?
Who does it usually affect?
What is the typical presentation? (4)
Medium size vasculitis
1) Cervical lymphadenopathy
3) strawberry tongue
4) desquamating rash
Complication: CORONARY ANEURYSMS