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
