Vasculitis Flashcards
What material is in each of the heart layers
Intimate = endothelial cells resting on BM Media = smooth-muscle Adventitia = connective-tissues
What is vasculitis
Inflammation of
small sized vessels
Medium sized vessels
OR COMBO
What is the aetiology of vasculitis
Unknown - most cases NOTTTTT infectious
Non-specific symptoms of vasculitis
Fever, fatigue, weight loss, myalgia
What does vasculitis preferentially involve?
Symptoms of organ ischaemia?
Vasculitis preferentially involves certain organs vascular beds
- Damage endotheliocyte all – >
expose subendothelial collagen – >
activate thromboplastin -> activate coag cascade ->
THROMBOSIS - Inflammation of wall -> healing –> fibrosis BV wall –> narrow lumen = decreased blood flow – >
organ ischaemia
Give two large vessel vasculitis
How do they present?
GreaT PaKi Buerger
GWanny Chemist
GreaT (large!!)
Giant cell/temporal arteritis
Takayasu arteritis
Present with loss of pulse/ stroke
Give two medium vessel vasculitis
How do they present?
GreaT PaKi Buerger
GWanny Chemist
Polyarteritis nodosa
Kawasaki
Present = vessel thrombosis + infarction/aneurysm
Give two small vessel vasculitis
How do they present?
GreaT PaKi Buerger
GWanny Chemist
Granulomatosis with polyangiitis = wegener
Churg Strauss
HEnoch Schonlein purpura
Microscopic Polyarteritis
Present = skin overlying vasculitis =
haemorrhagic raised painful @ palpation
Who does giant cell temporal Arteritis happen in?
Classic location of giant cell temporal arteritis?
Females
> 50
Branches of carotid
Temporal – headache
Ophthalmic – visual issues
Artery of jaw muscles - Jaw claudication
What other disease is giant cell temporal arteritis associated with
Polymyalgia rheumatica – flulike symptoms + joint and muscle pain
Increased ESR > 100
What does biopsy of giant cell temporal arteritis reveal
Inflamed vessel wall
Giant cells = granulomatous inflammation
Intimal fibrosis
For giant cell temporal arteritis what should you be wary of during biopsy
Treatment? What happens if you don’t treat?
Lesions are segmental
Only one area could be involved
@biopsy
- take long piece
- examine all of piece under a microscope
- if negative – doesn’t mean there is no disease = could have taken part of vessel where disease isn’t present
corticosteroids - without treatment = blindness
Explain how Takayasu arteritis differs from giant cell temporal Arteritis.
Treatment for Takayasu arteritis?
Giant cell temporal arteritis =
Females >50
Takayasu arteritis =
Young Asian female < 50
GCTA + Carotid branches
Takayasu Arteritis - branch points of Aorta
Granulomatous thickening + narrowing aortic arch
CORTICOSTEROIDS
What organs does polyarteritis nodosa involve
Most organs except lungs - NOT pul art!!
CNS vessels - NEURO dysfunction
Visceral art. - mesenteric vessels ab pain + melena
Renal vessels - HTN/renal damage
Necrotising skin lesions - cutaneous eruptions
In 30% of patients with polyarteritis nodosa say what serum surface antigen is present?
Hep B
Explain the pathogenesis of polyarteritis nodosa
Treatment for polyarteritis nodosa
Early lesions = transmural inflammation – whole vessel is inflamed with fibrinoid necrosis
Bumps = aneurysm
Bits in between bumps = fibrosis
After healing
End lesions = massive fibrosis – string of pearls
bumpy @ blood vessel (Nodosa = feels like a node)
Corticosteroids + CYCLOPHOSPHAMIDE
Who does Kawasaki disease happen in?
Asian kids < 4
FCETH DCL
fETCh CoLD
Erythematous rash @ palms/soles
Thrombosis/rupture cos of Coronary Art aneurysm
Cervical lymphadenitis
COnjunctivitis
Lips + oral mucosa strawberry tongue
Desquamating rash
How do you treat a kid with Kawasaki?
Aspirin –> inhibit Cox = Decrease THx A2 ->
Decreased aggregation -> destroy thrombus
What happens in buerger thromboangiitis
Intermittent claudication – >
bUeRGArS disease
B-- Ulceration E-- Raynaud of fingers Gangrene Auto-amputation R-- Superficial nodular phlebitis of leg veins
Who does buergers thromboangiitis happen in?
How do you treat this?
Heavy smokers < 40yrs
Stop smoking
What happens in the weCener granulomatosis
Necrotising granulomatous vasculitis @
Nasopharynx - sinusitis/nasopharyngeal ulcer
Lung - Hemoptysis/bilateral nodular lung infiltrate
Kidney - hematuria - rapidly progressive glomerulonephritis
What are the ANCA results for the small vessel Vasculitis
WeCener = c-ANCA - anti-proteinase 3
Micro. polyangiitis = p-ANCA - anti-myeloperoxidase
Churg Strauss = p-ANCA
What does biopsy revealed the wegener granulomatosis
Treatment?
Necrotising granuloma @ lung/upper airway
Corticosteroids + Cyclophophamide
How does microscopic polyangiitis differ from wegener?
Treatment?
No nasopharyngeal involvement
P – ANCA
No granuloma
pauci-immune glomerulonephritis
Palpable purpura
Corticosteroids + Cyclophophamide
Which vasculitis require corticosteroids and cyclophosphamide?
Which vasculitis requires corticosteroids only?
Which vasculitis require
smoking cessation +
IV Ig + aspirin
Polyarteritis nodosa, Wegener, microscopic polyang
Giant cell temporal arteritis, Takayasu
Burger disease, Kawasaki
Which vasculitis diseases are necrotising
Burger, Wegener, Microscopic polyangiitis,
Churg Strauss
What do you get Churg Strauss syndrome
GAPe PaPES
Granuloma necrotizing, Asthma, PEripheral eosinophilia
Palpable purpura, Peripheral neuropathy, Eosinophils, Sinusitis
What is the most common vasculitis in children?
Pathophys?
What is the classic presentation?
HEnoch-Schonlein Purpura
Upper respiratory infection – > XS IgA production ->
Arthralgia, GI pain/Melena, PALPABLE purpura
Which vasculitis are similar to each other
Giant cell temporal = Takayasu
Wegener = Microscopic Polyangiitis = Churg Strauss
