Flashcards in Vasculitis Deck (54)
What is vasculitis?
Inflammation of the blood vessel wall
What is the arterial wall comprised of?
three layers: endothelial intima, smooth muscle media, and connective tissue adventitia
What is the etiology for vasculitis?
usually unknown; most cases are not infectious.
What do the clinical features for vasculitis include?
1) Nonspecific symptoms of inflammation (e.g., fever, fatigue, weight loss, and myalgias) 2) Symptoms of organ ischemia—due to luminal narrowing or thrombosis of the inflamed vessels
Why are there symptoms of organ ischemia in vasculitis?
Its due to luminal narrowing or thrombosis of the inflamed vessels
How is vasculitis divided?
into large-, medium-, and small-vessel vasculitides
What does large-vessel vasculitis involve?
the aorta and its major branches.
What does medium-vessel vasculitis involve?
muscular arteries that supply organs.
What does small-vessel vasculitis involve?
arterioles, capillaries, and venules.
What are the large vessel vasculitis?
Temporal (Giant Cell) Arteritis and Takayasu Arteritis
What is temporal (Giant Cell) Arteritis?
Granulomatous vasculitis that classically involves branches of the carotid artery
What is the most common form of vasculitis in older adults (> 50 years)?
temporal (Giant Cell) Arteritis and usually affects females
How does temporal (Giant Cell) Arteritis present?
as headache (temporal artery involvement), visual disturbances (ophthalmic artery involvement), and jaw claudication. Flu-like symptoms with joint and muscle pain (polymyalgia rheumatica) are often present. ESR is elevated.
What does biopsy of temporal (Giant Cell) Arteritis reveal?
inflamed vessel wall with giant cells and intima fibrosis
How do the lesions appear?
They are segmental;
What does diagnosis of temporal (Giant Cell) Arteritis require?
biopsy of a long segment of vessel, and a negative biopsy does not exclude disease.
What is the treatment for temporal (Giant Cell) Arteritis?
it is corticosteroids; high risk of blindness without treatment
What is Takayasu Arteritis?
Granulomatous vasculitis that classically involves the aortic arch at branch points
Takayasu Arteritis presents in?
adults < 50 years old (classically, young Asian females) as visual and neurologic symptoms with a weak or absent pulse in the upper extremity ('pulseless disease'). ESR is elevated.
What is the treatment for Takayasu Arteritis?
it is corticosteroids
What are the medium vessel vasculitis?
Polyarteritis Nodosa, Kawasaki Disease, Buerger Disease
What is Polyarteritis Nodosa?
Necrotizing vasculitis involving multiple organs; lungs are spared.
How does Polyarteritis Nodosa classically present?
in young adults as hypertension (renal artery involvement), abdominal pain with melena (mesenteric artery involvement), neurologic disturbances, and skin lesions.
What is Polyarteritis Nodosa associated with?
In Polyarteritis Nodosa what types of lesions are present?
Lesions of varying stages are present.
What does the early lesion of Polyarteritis Nodosa consist of?
transmural inflammation with fibrinoid necrosis that eventually heals with fibrosis,
How does the early lesion of Polyarteritis Nodosa appear on imaging?
It produces a string-of-pearls appearance on imaging
What is the treatment for Polyarteritis Nodosa?
corticosteroids and cyclophosphamide; fatal if not treated
Who does Kawasaki Disease classically affect?
Asian children < 4 years old