Vascular Disease 3- Leah (3)- Vasculitis* Flashcards
Giant Cell Arteritis (GCA): what vessels are involved?
small –> large
esp those in the head (temporal, ophthalmic, vertebral)
occasionally the aorta= giant cell aortitis
In what vessels is polyarteritis nodosa observed?
small –> medium
kidneys, heart, liver/ GI
*SPARES LUNGS
Kawasaki disease involves what arteries?
small/ large
most commonly the coronaries
Churg Strauss involves what vessels?
small
kidney, skin, GI, ocassionally the heart
Wegener’s granulomatosis effects what vessels?
small and medium
Lung + upper airway*, and renal
Microscopic polyangitis effects what vessels?
Small (rarely large)
very wide distribution of lesions throughout body
*Does NOT spare the lung, unlike PAN
Thromboangiitis obliterans effects what vessels?
small/ medium
most commonly tibial and radial
Which two vaculidities are assc with nodular aneurysms?
- PAN
- Kawasaki
Which vasculidity is assc with fragmented neutros?
MPA
Which vasculidity is assc with micro-abscesses and gangrene? Where are the micro-abcesses found?
Thromboarteritis Obliterans:
Vasculitis causes multiple thrombi in vessel lumen,
micro-abcesses are found within the thrombi
Which four vasculidities are assc with segmental lesions?
What is the significance of this findings?
- GCA
- PAN
- CS
- TO
- Good People Can Tag Team*
- Larger samples (2-3 cm) must be taken when taking biopsies to screen for these conditions
In which layer of the vascular wall are granulomas found in giant cell arteritis?
What are two other histo findings associated with this disease?
How often does this disease have giant cells?
- granulomas in media
- intimal thickening
- elastic lamina fragmentation
- giant cells only 75% of the time
Polyarteritis nodosa histologic findings: (4)
- fibrinoid necrosis/ transmural segmented lesions
- simultaneous early + late lesions
- fibrous thickening (late lesions)
- aneurysmal nodules (early lesions)
Kawasaki histologic findings: (2)
- aneurysms
- transmural lesions and fibrinoid necrosis, but less than in PAN
Describe the lesions found in MPA (4)
- fibrinoid necrosis, transmural lesions
- all lesions in the same stage
- lesions typically pauci-immune (no Ig/ complexes)
- possible fragmented neutros
In addition to granulomas, fibrinoid necrosis, and transmural lesions found in Churg Strauss… what histo findings may be present? (2)
- eosinophils!!!!
- Also note that granulomas may be EXTRAVASCULAR
Special characteristic of granulomas found in thromboarteritis obliterans (2):
Two things patients may present with clinically?
- Fibrotic tissue may involve nerves and veins
- Nerve involvement = PAINFUL in late stage disease
*Clinical presentation: cold sensitivity in hands, instep claudication
Signs/ Symptoms of Giant Cell Arteritis (2):
What population is this seen in?
Treatment?
- elevated inflammatory markers
- palpable, painful, nodular vessels + H/A, blindness
- elderly esp in US + Europe (#1 elderly vasculitis)
- Give corticosteroids MEDICAL EMERGENCY
Findings (Classic Triad+2) + Cause of Death assc with PAN:
Age group assn?
TRIAD:
- rapid accelerating HTN
- abdominal Pain
- bloody stool
- ruptured aneurysms
- decreased perfusion injuries (ulceration, infarcts etc)
- CAUSE OF DEATH = renal vasculitis + malignant HTN (no glomerulonephritis!)
- young adults
Treatment for PAN:
-corticosteroids, cyclosporine ++ treat the HTN
Kawasaki Disease:
symptoms+ cause of death, course of disease, age group, treatment
CAUSE OF DEATH = ruptured aneurysms and MI "CRASH and BURN" -Conjunctivitis -Rash (lips, palms, soles) -Adenopathy (cervical, unilateral) -Strawberry tongue -Hand + foot edema/erythema -Burn = FEVER
Tx: ASN and IVIG
*Kids under 4yoa, classically Asians (Hence KAWASAKI!)
Churg Strauss key findings: (4)
- eosinophils
- asthma
- allergic rhinitis
- less than 50% will be MPO-ANCA +