7.1 Vasculitis Flashcards
Wegener’s granulomatosis
-clinical presentation
Middle aged male with:
- Nasal: sinusitis/nasopharyngeal ulceration
- Lungs: hemoptysis with bilateral nodular lung infiltrates
- Kidneys: rapidly progressive glomerulonephritis
- remember “weCener’s”
Churg-Strauss syndrome
- what is it
- what is characteristic of it
- necrotizing granulomatous vasculitis, with eosinophils. Aka allergic granulomatosis
- asthma, peripheral eosinophilia often present
- many organs affected
Takaysu arteritis
-what vessels classically involved
aortic arch branch points
which vessels most classically involved in Temporal Giant cell arteritis?
branches of carotid
Kawasaki’s disease
-clinical presentation (4 general symptoms)
- nonspecific signs, looks like viral infection in young child.
1. fever
2. conjunctivitis
3. erythematous rash of palms, soles
4. enlarged cervical lymph nodes (mucocutaneous lymph node syndrome)
Kawasaki’s disease
-treatment
- aspirin and IVIG
- yes, aspirin in a child even though Reye’s syndrome can occur in child with viral illness, which is what Kawasaki’s looks like.
- disease is usually self limited
microscopic polyangiitis
-what organs most affected
- necrotizing vasculitis, small vessels
- multiple organs, esp lung and kidney
Takaysu arteritis
-symptoms
- visual and neurologic symptoms
- weak/absent pulse in upper extremity (“pulseless disease”)
Kawasaki’s disease
-what is the big risk/complication?
-coronary artery involvement, can lead to thrombus (MI) and aneurysm
p-ANCA vs c-ANCA:
-what diseases do these differentiate?
p-ANCA: microscopic polyangiitis and Churg-Strauss syndrome
c-ANCA: Wegener’s Granulomatosis (“WeCener’s”)
Why do you see organ ischemia in vasculitis?
-2 mechanisms
- luminal narrowing of vessels. inflammation, fibrosis
- thrombosis of inflamed vessels
Buerger’s disease
-clinical presentation
aka Thromboangiitis obliterans
- necrotizing vasculitis and thrombosis involving digits–Raynaud’s phenomenon often present
- painful ischemia, peripheral pulses diminished
- ulceration, gangrene, autoamputation of fingers, toes.
Wegener’s granulomatosis
-classic population?
middle aged male
Wegener’s granulomatosis
- lab test used for diagnosis
- what do you see on biopsy?
- tx
‘WeCener’s’
- c-ANCA
- large necrotizing granulomas with adjacent vasculitis
- Cyclophosphamide
polyarteritis nodosa
-treatment
corticosteroids and cyclophosphamide
-fatal if untreated
what is ANCA test?
what is p-ANCA and c-ANCA?
- ANCA: anti-neutrophil cytoplasmic antibody
1. put neutrophil on slide
2. Put pt’s serum on slide
3. if serum has Ab against neutrophil, you will see ANCAs
c-ANCA: cytoplasmic Ab (cytoplasm away from nucleus)
p-ANCA: pernuclear Ab (next to nucleus)
polyarteritis nodosa
-what blood test finding associated with?
serum HBsAg
(hep B surface Ag)
temporal giant cell arteritis:
-ESR level?
- ESR is elevated (>100)
- often see flu-like symptoms in pt
microscopic polyangiitis vs Churg-Strauss syndrome:
-how to differentiate? (3 things)
Both are p-ANCA
- C-S has granulomas
- C-S often has asthma
- C-S often has peripheral eosinophilia
Henoch-Schonlein purpura
- course of disorder
- tx
- typically self-limiting without tx
- Tx with corticosteroids if severe
Henoch-Schonlein purpura
-how does it present?
- Purpura: palpable purpura on buttocks, legs
- GI: GI pain/bleeding
- Kidneys: hematuria (IgA nephropathy)
temporal giant cell arteritis
-what does biopsy reveal?
- See inflamed vessel wall with giant cells and intimal fibrosis.
- lesions are segmental, so requires biopsy of long section of vessel
- absence of lesion still doesn’t rule it out
microscopic polyangiitis
-how is presentation different from Wegener’s granulomatosis (2 things)
- microscopic polyangiitis is similar to Wegener’s granulomatosis except:
1. no nasal pharyngeal involvement
2. granulomas absent.
Wegener’s granulomatosis
- what is it
- what organs involved?
-necrotizing granulomatous vasculitis, small vessels
Organs:
- nasopharynx
- lungs
- kidneys
Henoch-Schonlein purpura
- what is it
- mech
- when do pts typically get it
- Vasculitis of small vessels from depositions of IgA complexes
- typically occurs after upper respiratory infection, which creates IgA, which deposits throughout body
polyarteritis nodosa
-classic presentation (4 symptoms)
- HTN from renal artery
- Abdominal pain with melena (mesenteric a)
- neurologic disturbances
- skin lesions
polyarteritis nodosa
-what organs are affected, which ones not?
-all organs potentially affected except lungs
Vasculitis in general:
-symptoms
- nonspecific symptoms of inflammation (fever, fatigue, weight loss, mylagias)
- organ ischemia, from:
1. luminal narrowing (inflammation, then fibrosis)
2. thrombosis of inflamed vessels
What syndrome is Kawasaki’s syndrome also called, and why?
mucocutaenous lymph node syndrome
- mucus membrane inflammation,
- enlarged lymph nodes, esp cervical
what is the most common form of vasculitis in older adults (>50)
temporal giant cell arteritis
Vasculitis:
- name disorders in each category
1. large (2)
2. med (3)
3. small (4)
- large: Temporal Giant Cell arteritis, Takayasu’s arteritis
- medium: Polyarteritis nodosa, Kawasaki’s disease, Buerger disease
- small: Wegener’s granuomatosis, microscopic polyangiitis, churg-strauss syndrome, henoch-schonlein purpura
Raynaud’s phenomenon:
- color changes in fingers?
- what are some disorders secondary Raynaud’s is associated with?
- white, blue (vessels dilate to retain blood), then red (reperfusion)
- SLE, scleroderma, Buerger’s disease, atherosclerosis
Takaysu arteritis
-treatment
corticosteroids
Takaysu arteritis
-classic population
-esp young Asian females
polyarteritis nodosa
-affected population
-classically youg adults
Kawasaki’s disease
-classic population affected
-asian children
Takaysu arteritis
-it’s pretty much the same as giant cell arteritis, with what 2 big exceptions?
- population: 50 in giant cell arteritis
- location: aortic arch branches, instead of carotid a branches
polyarteritis nodosa
-describe pattern of lesions
“string of pearls”
- lesions of varying stages at once:
- early lesions of transmural inflammation with fibrinoid necrosis–this causes stretching, aneurysm of vessel (‘pearls’)
- late lesions that are healed, with fibrosis (‘string’)
temporal giant cell arteritis:
treatment
- corticosteroids
- treat immediately if suspected–don’t wait for biopsy results. Can lead to blindness!
Buerger’s disease
-what risk factor associated with?
- heavy smoking. highly associated
- Tx is to stop smoking
“pulseless disease”
-what is this referring to?
Takaysu arteritis.
-weak/absent pulses in upper extremity
temporal giant cell arteritis:
-symptoms (classic 3)
- headache (temporal a)
- visual disturbances (ophthalmic a)–can lead to blindness!
- jaw claudication
- flu like symptoms often present, ESR elevated
microscopic polyangiitis
-tx
-corticosteroids, cyclophosphamide
