7.1 Vasculitis Flashcards
What is vasculitis?
Inflammation of blood vessel wall
What are the symptoms of vasculitis?
- Nonspecific
- Organ ischemia
What does large vessel vasculitis tend to affect?
Aorta and its branches
What are the types of large vessel vasculitides?
- Giant Cell Arteritis (GCA)
- Takayasu’s Arteritis
What is the typical patient of GCA?
Older females (>50)
Tends to affect Caucasians
What is the classic location of GCA?
Branches of the carotid
- temporal artery
- ophthalmic artery causing visual disturbance
What is seen on LM of GCA?
Granulomatous vasculitis with giant cells and intimal fibrosis
Why must a long piece be taken for GCA biopsy?
The lesions are segmental - negative biopsies may not exclude the disease
How does GCA cause ischemia?
Fibrosis in between the intimal and the media that leads to narrowing of the lumen which decreases perfusion
What is the treatment for GCA?
Corticosteroids - treat ASAP
What is the typical patient of Takayasu’s Arteritis?
Adults less than the age of 50 - typically an Asian female
What does LM of Takayasu’s Arteritis show?
Granulomatous vasculitis
What vessels does Takayasu’s Arteritis tend to affect?
Aortic arch at the branch points
What are some clinical signs of Takayasu’s Arteritis?
- Weak or absent pulse in the upper extremities
- ESR is elevated
What is the treatment for Takayasu’s Arteritis?
Corticosteroids
What does medium vessel vasculitis tend to affect?
Muscular arteries that supply organs
What are the types of medium vessel vasculitis?
- Polyarteritis Nodosa (PAN)
- Kawasaki Disease
- Buerger Disease
What are the effects of polyarteritis nodosa?
Necrotizing vasculitis that involves multiple organs
What organ is spared by PAN?
Lungs
What is the characteristic finding of PAN on imaging?
“String on pearls” - aneurysms between fibrous areas of the vessel
What are some of the characteristics of PAN?
- Fibrinoid necrosis
- Varying stages of disease along the same artery
What is the the treatment for PAN?
Corticosteroids
Cyclophosphamide
-Fatal if not treated
What is the typical patient of Kawasaki Disease?
Asian children less than the age of 4
What are the presentations of Kawasaki Disease?
Fever
Conjunctivitis
Erythematous rash of palms and soles - “motorcycle”
What is the preferential artery involved in Kawasaki Disease?
Coronary Artery
What are the complications of Kawasaki Disease?
- MI
- Aneurysm and rupture
What is the treatment of Kawasaki Disease and why is it unique?
Aspirin and IVIG
- Normally you would NEVER give a child aspirin as it risks Reye’s Disease
How does aspirin treat Kawasaki Disease?
Aspirin prevents TXA2 to prevent thrombus formation in the coronary artery
What is Buerger Disease?
Necrotizing vasculitis of the digits that presents with ulcers and gangrene
What is Buerger Disease associated with?
Smoking
What is the treatment for Buerger Disease?
Cessation of smoking
What is a sign of Buerger Disease?
Raynaud’s Phenomenon
What does small vessel vasculitis affect?
Arterioles, capillaries and venules
What is Wegener’s Granulomatosis?
Necrotizing granulomatous vasculitis that involves the nasopharynx, lungs and kidneys
What is the pathogenesis of Wegener’s Granulomatosis?
c-ANCA
What is the typical patient of Wegener’s Granulomatosis?
Middle aged male with sinusitis or nasopharyngeal ulceration and hemoptysis with hematuria
What is the treatment for Wegener’s Granulomatosis?
Cyclophosphamide
Corticosteroids
What is Microscopic Polyangiitis?
Necrotizing vasculitis with lung and kidney involvement
What are the difference between Microscopic Polyangiitis and Wegener’s?
- No nasopharyngeal
- No granulomas
- pANCA instead of cANCA
What is the treatment for Microscopic Polyangiitis?
Cyclophosphamide
Corticosteroids
What is Churg-Strauss Syndrome?
Necrotizing granulomatous vasculitis with eosinophils
What is the pathogenesis of Churg-Strauss Syndrome?
pANCA
What are the differences between Churg-Strauss Syndrome and Microscopic Polyangiits?
CS wil have:
- Granulomas
- Eosinophilia
- Asthma
What is the most common vasculitis in children?
Henoch-Shonlein Purpura
What is the pathogenesis of Henoch-Shonlein Purpura?
Deposition of IgA following an URI due to excessive production
What are the signs of Henoch-Shonlein Purpura?
- Palpable purpura on buttocks and legs
- Hematuria with IgA nephropathy
What does Henoch-Shonlein Purpura tend to follow?
Respiratory tract infection
What is the treatment for Henoch-Shonlein Purpura?
Self-limiting - use steroids only in severe cases
