Vasculitis Flashcards
how do you divide vasculitis?
Large, medium and small
what are the types of large vessel vasculitis
Takayasu’s
Giant cell
What are the types of medium vessel vasculitis
Kawasaki’s
Polyarteritis nodosa
Behcet’s disease
Churg - STrauss (P-ANCA positive)
Which medium vessel vasculitis is negative for blood markers?
Kawasaki’s
Polyarteritis nodosa
Behcet’s disease
Which medium vessel vasculitis is positive for blood marker and which marker?
Churg - Strauss (P-ANCA)
What are the types of small vessel vasculitis?
Henoch - Schonlein purpura Essential cryoglobulinemia arteritis of connective tissues disease (Scl, SLE, RA, MCTD) Wegener's granulomatosis (C-ANCA) Microscopic polyangiitis (P-ANCA)
which small vessel vasculitis are blood test negative?
Henoch - Schonlein purpura
Essential cryoglobulinemia
Arteritis of connective tissue disease (Scl, SLE, RA, MCTD)
which small vessel vasculitis are blood test positive?
Wegener's granulomatosis (C-ANCA) Microscopic polyangiitis (P-ANCA)
Takayasu - Ueno Classification class 1?
localized to the arch of the aorta and branches
Takayasu - Ueno Classification class 2a?
ascending aorta, arch and branches
Takayasu - Ueno Classification class 2b?
ascending aorta, arch and branches, descending thoracic aorta
Takayasu - Ueno Classification class 3.
descending thoracic aorta, abdominal aorta +/- renal arteries
Takayasu - Ueno Classification class 4?
abdominal aorta +/- renal arteries
Takayasu - Ueno Classification class 5?
ascending aorta, arch and branches, descending thoracic aorta and abdominal aorta +/- renal arteries
What does C or P mean in Takayasu - Ueno classification?
C- coronary
P - pulmonary
what’s Reynauld’s disease?
idiopathic, vasospastic, bilateral (Primary)
at room temperature patients have normal PVR
needs cold immersion test
What’s Reynauld’s syndrome?
secondary to occlusive pathology or connective tissue disease
patients have abnormal PVRs at room temperature
what’s the antibody for Scleroderma?
specled ANA
what’s the antibody for SLE?
homogenious ANA
what’s the antibody for CREST
Anticentromere Ab
What’s the antibody for Sjogrens
SS Ab
Diagnostic criteria for Giant cell Arteritis
- age >50
- localized headache
- temporal artery tenderness
- ESR >50
- temporal artery biopsy + for giant cell arteritis
Diagnostic criteria for Behcet disease?
- major - oral ulcers
- minor (needs two)
a) genital ulcers
b) eye lesions (uveitis 80%)
c) skin - erythema nodosum
d) venous thrombosis (50%)
e) arterial pathology: aneurysms (AAA>pulmonary>femoral > popliteal>brachial>iliac) or occlusive disease
f) other: pericarditis, arthritis, GI ulcers, CNS
What are the aneurysms in Behcet disease?
aorta > pulmonary > femoral > popliteal > brachial > iliac
Characteristics of Poliarteritis Nodosa?
systemic necrotizing vasculitis abdominal pain in young adults multiple mesenteric aneurysms increase ESR, normal ANCA 30% have positive hepatitis B, associated with HIV 2:1 male
Characteristics of Kawasaki disease?
high fever, remittent for 2 weeks bilateral conjunctival inflammation strawberry tongue, swollen lips erythema of palmes and soles 20-30% coronary aneurysms
Coronary and brachiocephalic aneurysms in 25% of untreated patients
MI and arrhythmia most frequent cause of pain
Can be prevented with ASA and IV IG during acute phase
Aneurysms <4mm universally egress and >8 mm don’t regress
Characteristics of Churg - STrauss?
- alergic phase - cold, asthma
- eosinophilia with infiltrates (PNA, gastrienteritis, neuropathy)
- vasculitis - most common coronary
- P-ANCA positive
- proximal vessel involvement
Wegener’s disease characteristics?
necrosing granulomatosus vasculitis
kidney and upper respiratory tract lesion
digital ischemia
C-ANCA 90%
Differential diagnosis of digital ischemia
- ANCA + : microscopic polyangiitis and Wegener’s
- ANCA -: CTD, cryoglobulinemia
- Trauma: vibration, frost bite
- Embolic
Types of fibromuscular displasia
- intimal fibroplasia
- medial fibroplasia
- perimedial fibroplasia
- adventitial fibroplasia/hyperplasia
which type of FMD is most common
medial fibroplasia
characteristics of intimal fibroplasia FMD
1:1 M:F, 10%
young
ringlike focal stenosis or long irregular tubular stenosis
characteristics of medial fibroplasia FMD
adolescent, 80%
9:1 F:M
string of beads, distal 2/3 of renal artery
characteristics of perimedial fibroplasia FMD
young girls and women, 5%
also string of beads but diameter of bead does not exceed proximal vessel
characteristics of adventitial fibroplasia FMD
1:1 M:F
unifocal long stenosis
Takayasu’s therapy
1mg/kg for 1 - 3 months
medical therapy for Buerger’s
- antiplatelets
- calcium channel blockers
- prostaglanding analogues (Iloprost)
segmental arterial mediolysis
simmilar to FMD but affects splanchnic vessels of middle aged and elderly individuals, can cause hypovolemic shock, PSA formation
Moyamoya disease
“puff of smoke”
cerebral proliferative angiopathy
surgical revascularization for occluded carotid is beneficial in this condition (external carotid to internal carotid bypass or superficial temporal artery to MCA bypass)
FMD in children
focal intimal fibroplasia
Reynald’s disease duplex
DPG wave with somewhat smaller upstroke with sharp anacrotic notch and dicrotic notch located unusually high
Williams syndrome
elfin features of face
supravalvular aortic stenosis
hypercalcemia
mild intellectual disability
Loeys - Dietz syndrome
aortic aneurysms vessel tortuosity craniofacial abnormality bifid uvula and cleft palate hypertension skeletal malformation food alergies caused by mutation of TGF beta
How much FMD in the neck is bilateral
65%
For much FMD in the neck is associated with intracranial aneurysms
30%
Diagnostic criteria for giant cell arteritis
At least theee of the following: 1. Age>50 2. Sudden onset of localized headache 3 temporal tenderness or decreased pulse 4. ESR >50 5. Histologic findings of inflammation
Surgical indications for Loeys - Dietz
Aortic root >4 cm
Descending thoracic aorta >5cm
Infrarenal aorta >4cm
What’s the prophylaxis for Loeys Dietz syndrome
Angiotensin II receptor inhibitor
Pedowitz criteria for chronic exertions compartment syndrome
Normal resting compartment pressure of >15mmHg
>30mmHg 1 min post exercise
>20mmHg 5 min post exercise