Vasculitides (STEP 1 Book) Flashcards
What are the large vessel vasculitis (2)?
Temporal (giant cell) arteritis
Takayasu arteritis
Temporal arteritis presents as?
Unilateral headache, jaw claudication
Irreversible blindness due to ophthalmic artery occlusion
Polymyalgia rheumatica association
Temporal arteritis most commonly goes after which artery?
Carotid artery
What type of inflammation involved in Temporal arteritis?
Focal granulomatous inflammation with increase in ESR
How do you treat Temporal Arteritis?
High dose corticosteroids prior to artery biospy
Takayasu arteritis characterized by?
“Pulseless disease” (Weak upper extremity pulses)
Ocular disturbances
Fever
Skin nodules
Lab findings of Takayasu arteritis?
Granulomatous thickening and narrowing of aortic arch and proximal great vessels
Increased ESR
How do you treat Takayasu?
Corticosteroids
What are the medium vessel vasculitis (3)?
Polyarteritis nodosa Kawasaski disease Berger disease (Thromboangiitis obliterates)
Who does Polyarteritis nodosa occur in?
Young adults
What does Hepatitis B have to do with Polyarteritis Nododa?
30% of patients have Hep b seropositivity
What vessels does Polyarteritis nodosa affect?
Renal and visceral vessels, no pulmonary arteries
What causes the vasculitis in Polyarteritis nodosa?
Immune complex mediated
What type of necrosis in Polyarteritis nodosa?
Fibrinoid necrosis of arterial wall
What affect does Polarteritis nodosa have on the kidney?
Innumerable renal micro aneurysms and spasms
How do you treat Polyarteritis nodosa?
Corticosteroids
Cyclophosphamide
Who does Kawasaki disease occur in?
Asian children less than 4 years old
CRASH and Burn mnemonic for Kawasaki?
Conjunctival injection Rash Adenopathy Strawberry Tongue Hand foot changes Burn = fever
Cause of death for Kawasaki cases?
Coronary artery aneurysms thrombosis or rupture
How do you treat Kawasaski
IV immunoglobulin
Aspirin
Who is Buerger disease in?
Heavy smokers less than 40
Danger of Buerger disease?
Intermittent claudication leads to gangrene
Autoamputation of digits
Superficial nodular phlebitis
Raynauds
How do you treat Buerger disease?
Have them stop smoking
What are the small vessel vasculitis (4)?
Granulomatosis with polyangitis (Wegener)
Microscopic polyangitis
Eosinophilic granulomatosis with polyangitis (Churg-Strauss)
Henoch-Schonlein purpura
What is Wegener vasculitis triad?
Focal necrotizing vasculitis
Necrotizing granulomas in lung/upper airway
Necrotizing glomerulonephritis
Wegener’s associated with what antibody?
Anti-proteinase 3
c-ANCA
Treatment for Wegener’s?
Cyclophosphamide
Corticosteroids
What is Microscopic Polyangitis?
Necrotizing vasculitis commonly involving lungs, kidneys, and skin with glomerulonephritis and palpable purpura
Microscopic is similar to Wegener’s minus what feature?
Nasopharyngeal involvement, Microscopic has none
No granulomas
Antibod associated with Microscopic polyangitis?
Anti-myeloperoxidase
p-ANCA
How do you treat Microscopic polyangitis?
Cyclophosphamide
Corticosteroids
What does Eosinophilic granulomatosis with polyangitis cause?
Asthma, sinusitis, skin nodules, peripheral neuropathy
Heart, GI, kidneys (glomerulonephritis)
How does Eosinophillic granulomatosis present?
Granulomatous necrotizing vasculitis with eosinophilia
What antibody does Eosinophilic granulomatosis associate with?
p-ANCA
Increase in IgE
Henoch Schonlein is what?
Most common childhood systemic vasculitis that follows URI
Classic triad of Henoch Schonlein?
Palpable purpura on ass/legs
Arthralgia
GI abdominal pain
What causes Henoch Schonlein?
Vasculitis secondary to IgA immune complex deposition
What other disease does Henoch schonlein associate with?
Berger disease (IgA neuropathy)
Easy way to remember treatments for vasculitis?
Only one that isn’t corticosteroids is Kawasaki which is immunoglobulin and aspirin