Vasculitides Flashcards
Name the Large-Vessel Vasculitides
Giant Cell (Temporal) Arteritis Takayasu Arteritis
Name the Medium-Vessel Vasculitides
Polyarteritis Nodosa Kawasaki Disease Burger Disease (Thromboangiitis Obliterans)
Name the Small-Vessel Vasculitides
Granulomatosis with Polyangiitis (Wegener)
Microscopic Polyangiitis
Eosinophilic Granulomatosis with polyangiitis (Churg-Strauss)
Henoch-Schonlein Purpura
Medium-vessel vasculitis involves what type of arteries? HY
muscular arteries that supply organs
Etiology of most vasculitis is what?
unknown and not infectious
What arteries does Giant Cell Arteritis usually involve?
branches of the carotid artery
what type of vasculitis is Giant Cell Arteritis?
Granulomatous (always with giant cell)
What is the most common form of vasculitis in older adults?
Giant Cell Arteritis
Giant Cell Arteritis usually effects what population?
usually affects older females
What is polymyalgia rheumatica and what vasculitis is associated with it?
flu-like symptoms with joint and muscle pain
Giant Cell Arteritis
What are the issues that arise from the arteries involved in giant cell arteritis?
headache - temporal
blindness - ophthalmic
jaw claudication
What blood test is elevated in Giant Cell Arteritis?
ESR > 100
Upon biopsy what do you see in giant cell arteritis?
inflamed vessel wall with giant cells and intimal fibrosis (focal granulomatous inflammation)
How do you treat Giant Cell Arteritis?
corticosteroids
Complication from not treating Giant Cell Arteritis? HY
high risk of blindness
What type of vasculitis is Takayasu and what arteries does is classically involve?
granulomatous vasculitis classically affecting aortic arch at branch points
Who gets Takayasu arteritis?
young Asian females
What arteritis is known as pulseless disease and what does that present with?
Takayasu
weak or absent pulse in upper extremity
What are some signs and symptoms of Takayasu?
flu-like symptoms with neurological and ocular disturbances along with a weak or absent upper extremity pulse
What blood test is elevated in Takayasu?
ESR
What is the treatment for Takayasu?
corticosteroids
Polyarteritis Nodosa gets signs and symptoms in every organ except what?
lungs
What presents in young adults as hypertension, abdominal pain with melena, neurologic disturbances, and skin lesions?
Polyarteritis Nodosa
What serotype is positive in Polyarteritis Nodosa? (HY)
serum HBsAg (Hep B)
Early vs Late lesions in Polyarteritis Nodosa?
early lesions consists of transmural inflammation with fibrinoid necrosis
late lesions heals with fibrosis
What vasculitis has strings-of-pearls appearance and what causes them? HY
Polyarteritis Nodosa?
Early lesions become aneurysms between the late fibrotic lesions
What is the prognosis of Polyarteritis Nodosa?
fatal if left untreated
What is the treatment for Polyarteritis Nodosa?
Corticosteroids and Cyclophosphamide
What is the immune pathogenicity of Polyarteritis Nodosa?
immune complex mediated (Type III Hypersensitivity)
Who gets Kawasaki Disease?
Asian children
What is one way to remember who gets Kawasaki Disease?
think of a young asian child riding a kawasaki bike - palms and soles affected
CRASH and Burn mnemonic for Kawasaki Disease?
Conjunctival injection Rash (polymorphous -> desquamating) Adenopathy (cervical) Strawberry tongue (oral mucositis) Hand-Foot erythematous rash fever
Kawasaki Disease commonly can affect what arteries with what complications? HY
Coronary artery involvement leading to (1) thrombosis with MI and (2) aneurysm with rupture
Treatment of Kawasaki Disease?
aspirin and IVIG
Another name for Beurger Disease
Thromboangiitis Obliterans
Who gets Thromboangiitis Obliterans (Beurger)?
heavy smokers, it is a smokers disease
Males
What is often present in Thromboangiitis Obliterans (Beurger)? HY
Raynaud Phenomenon
What type of vasculitis is Thromboangiitis Obliterans (Beurger)?
necrotizing vasculitis of digits
How does Thromboangiitis Obliterans (Beurger) present?
gangrene, ulceration, and autoamputation of fingers and toes
Treatment for Thromboangiitis Obliterans (Beurger)?
smoking cessation
What is another name for Wegeners?
Granulomatosis with polyangiitis
What type of vasculitis is Wegeners?
necrotizing granulomatous vasculitis
Where does Wegeners commonly affect?
Nasopharynx, Lungs, and Kidneys
What is the We”C”ener’s mnemonic? HY
lots of C’s with this disease
C shape distribution of disease
C-ANCA (PR3-ANCA)
Cyclophosphamide and Corticosteroids treatment
What vasculitis is classically seen in a middle aged male presenting with sinusitis, nasopharyngeal ulcers, hemoptysis, bilateral nodular lung infiltrates, and hematuria?
Granulomatosis with Polyangiitis (Wegeners)
Why do Wegeners patients get hematuria? HY
rapidly progressive necrotizing glomerulonephritis
What blood marker correlates with disease activity in Wegeners?
C-ANCA/PR3-ANCA
What does biopsy in Wegeners show?
large necrotizing granulomas with adjacent necrotizing vasculitis
What is the treatment for Wegeners? HY
Cyclophosphamide and corticosteroids
Prognosis of Granulomatosis with polyangiitis?
relapses are common
What is PR3-ANCA?
anti-proteinase-3
What vasculitis is similar in presentation to Wegeners but lacks nasopharyngeal involvement and has no granulomas?
Microscopic Polyangiitis
What kind of vasculitis is Microscopic Polyangiitis and what organ does it involve?
Necrotizing vasculitis involving especially lung, kidney and skin
How does Microscopic Polyangiitis present?
pauci-immune glomerulonephritis and palpable purpura
What blood marker correlates with disease activity in Microscopic Polyangiitis?
P-ANCA/MPO-ANCA
How do you differentiate between Wegeners and Microscopic Polyangiitis?
Microscopic Polyangiitis has no nasopharyngeal involvement and no granulomas
How do you treat Microscopic Polyangiitis?
corticosteroids and cyclophosphamide
What kind of vasculitis is Churg-Strauss and what organs are involved?
Granulomatous, Necrotizing vasculitis with eosinophilia involving especially lungs and heart
What vasculitis presents with asthma?
Churg-Strauss (Eosinophilic Granulomatosis with Polyangiitis)
What vasculitis presents with peripheral neuropathy leading to wrist and foot drop?
Churg-Strauss (Eosinophilic Granulomatosis with Polyangiitis)
What blood marker correlates with disease activity in Churg-Strauss?
P-ANCA/MPO-ANCA
How do you differentiate between Microscopic Polyangiitis and Churg-Strauss?
both have MPO/P-ANCA
Microscopic Polyangiitis has no asthma and no granulomas
Churg Strauss has granulomas, eosinophilia, and asthma
What type of kidney involvement is there in Churg-Strauss?
pauci-immune glomerulonephritis
What does pauci-immune mean?
vasculitis that is associated with minimal evidence of hypersensitivity upon immunofluorescence.
What is the most common vasculitis in children? HY
Henoch Schonlein Purpura
What does Henoch Schonlein Purpura occurs following what?
upper respiratory tract infection
What is Henoch Schonlein Purpura secondary to? HY
IgA immune complex deposition
Pathogenesis of Henoch Schonlein Purpura HY
Upper respiratory infection leads to high IgA due to mucosal involvement –> accumulation of IgA –> IgA complex deposition –> vasculitis
What is a major complication of igA deposition in Henoch Schonlein Purpura? HY
hematuria due to IgA nephropathy (Berger Disease)
What sign occurs in Henoch Schonlein Purpura due to inflammation? HY
PALPABLE purpura on buttocks and legs
Classic triad in Henoch Schonlein Purpura?
palpable purpura on buttocks/legs
Arthralgias
abdominal pain
Treatment for Henoch Schonlein Purpura?
self limited disease but may recur, treat with steroids if severe
What vasculitis presents with melena?
Polyarteritis Nodosa
