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
How does Kawasaki disease present?
with nonspecific signs including fever, conjunctivitis, erythematous rash of palms and soles, and enlarged cervicai lymph nodes
In Kawasaki disease involvement of what is common and what does this usually lead to?
Coronary artery involvement is common and leads to risk for (1) thrombosis with myocardial infarction and (2) aneurysm with rupture.
What is the treatment for Kawasaki disease?
It is aspirin and IVIG; disease is self-limited.
What is Buerger Disease?
Necrotizing vasculitis involving digits
How does Buerger Disease?
Presents with ulceration, gangrene, and autoamputation of fingers and toes
In Buerger Disease, what is often present?
Buerger Disease is highly associated with what patient behavior?
heavy smoking; treatment is smoking cessation.
What are the small vessel vasculitis?
Wegener Granulomatous, microscopic polyangiitis, Churg-Strauss Syndrome, Henoch-Schönlein Purpura
What is Wegener Granulomatosis?
Necrotizing granulomatous vasculitis involving nasopharynx, lungs, and kidneys
What is the classic presentation for Wegener Granulomatosis?
the classic presentation is a middle-aged male with sinusitis or nasopharyngeal ulceration, hemoptysis with bilateral nodular lung infiltrates, and hematuria due to rapidly progressive glomerulonephritis.
In Wegener Granulomatosis how can you gauge disease activity?
Serum c-ANCA levels correlate with disease activity,
What does Wegener Granulomatosis Biopsy reveal?
large necrotizing granulomas with adjacent necrotizing vasculitis
What is the treatment for Wegener Granulomatosis?
it is cyclophosphamide and steroids; relapses are common,
What is microscopic polyangiitis?
Necrotizing vasculitis involving multiple organs, especially lung and kidney
What is the presentation for microscopic polyangiitis?
It is similar to Wegener granulomatosis, but nasopharyngeal involvement and granulomas are absent.
How can you gauge microscopic polyangiitis disease activity?
Serum p-ANCA levels correlate with disease activity
What is the treatment for microscopic polyangiitis?
It is corticosteroids and cyclophosphamide; relapses are common.
What is Churg-Strauss Syndrome?
Necrotizing granulomatous inflammation with eosinophils involving multiple organs, especially lungs and heart
In Churg-Strauss Syndrome what else are also present in addition to the necrotizing granulomatous inflammation?
Asthma and peripheral eosinophilia are often present.
How could you gauge Churg-Strauss Syndrome?
Serum p-ANCA levels correlate with disease activity.
What is Henoch-Schönlein Purpura?
Vasculitis due to IgA immune complex deposition
What is the most common vasculitis in children?
How does Henoch-Schönlein Purpura present?
with palpable purpura on buttocks and legs, CI pain and bleeding, and hematuria (IgA nephropathy);
Henoch-Schönlein Purpura usually occurs following what?
an upper respiratory tract infection