Vasculitides Flashcards
What is the most common arteritis in adults?
temporal (giant cell) arteritis
What is the most common arteritis in children?
Henoch-Schönlein purpura
What is temporal (giant cell) arteritis?
What vessels does it typically effect and what changes occur in the vessel?
granulomatous, large vessel vasculitis
Vessels:
-carotid branches (cranial arteries) , particularly temporal artery
Changes:
- segmental granulomatous changes, multinucleated giant cells
- fibrosis
What is the clinical presentation of temporal (giant cell) arteritis?
Constitutional Sx:
- fever, malaise, weight loss
- headache (temporal A.; actually occurs along artery which may also be TTP)
- visual changes (ophthalmic A.); **sudden loss of vision**
- jaw claudication (facial A.)
What is the epidemiology of temporal (giant cell) arteritis?
- more common in females
- older adults (>50)
- european descent
What are diagnostic features of temporal (giant cell) arteritis?
-segmental granulomatous changes noted on temporal A. biopsy (because of segemental nature, false negative biopsy can occur)
-elevated ESR
What is the treatment and prognosis for temporal (giant cell) arteritis?
-corticosteroids upon ***suspicion of disease*** to prevent blindness (even before biopsy)
What are major complications of temporal (giant cell) arteritis?
- sudden onset, potentially permanent blindness; this is why you treat upon suspicion before biposy confirmation
- aortic aneurysm
- ischemic stroke
What other condition is often present with temporal (giant cell) arteritis?
-polymyalgia rheumatica (arthralgia and myalgia)
What is Takayasu arteritis?
What vessels does it typically effect and what changes occur in the vessel?
granulomatous, large vessel vasculitis
Vessels:
-aortic arch at branching points (crainal and upper ext As.)
Changes:
- smooth tapered stenosis
- granulomatous changes of aortic arch, multinucleated giant cells
- fibrosis
What is the epidemiology of Takayasu arteritis?
- more common in females
- adolescents to mid adults (15-45)
- more common Asians (Takayasu is Japanese -> Asian)
“young asian women”
What is the clinical presentation of Takayasu arteritis?
Constitutional Sx:
- fever, malaise, weight loss
- decreased radial/brachial pulses (“pulseless disease”)
- vision changes; “copper-wiring” retinopathy
- HTN (renal A.)
What are diagnostic features of Takayasu arteritis?
- smooth stenosis on angiography
- elevated ESR
What is the treatment and prognosis for Takayasu arteritis?
- corticosteroids
- typically regresses but aquired permanent changes (vision and neuro deficits) will remain
What is the main differentiating factor between temporal arteritis and Takayasu arteritis?
age; takayasu <50 and temporal >50
- very similar with constitutional s/x, visual changes, and neuro s/x
- very similar histology
What is polyarteritis nodosa?
What vessels does it typically effect and what changes occur in the vessel?
necrotizing systemic medium vessel arteritis
Vessels:
- muscular, medium arteries suppling most organs
- ***lungs spared***
Changes:
- transmural inflammation w/ fibrinoid necrosis (early) -> fibrous aneurysms (“string of pearls”; hence nodosa) (late)
- multiple, diffuse lesions (hence polyarteritis) found in varying stages
What is the clinical presentation of polyarteritis nodosa?
Constitutional Sx:
-fever, malaise, weight loss
Symptoms are manifestaions of organ ischemia** and **variable
- ulcers and gangrene
- HTN (renal A.)
- abdominal pain with melena (mesenteric As.)
- mononeuritis multiplex (foot drop)
What is the epidemiology of polyarteritis nodosa?
-more common in males
-young adults
What are diagnostic features of polyarteritis nodosa?
- multiple microaneurysms “string of pearls” on angiography
- fibrinoid necrosis on biopsy
-positive hepatitis B
What other condition is often present with polyarteritis nodosa?
hepatitis B infection; positive for HBsAg (hepaitits B surface Ag
What is the treatment and prognosis for polyarteritis nodosa?
- corticosteroids
- cyclophosphamide
**fatal if not treated**