Chapter 139 - Takayasu Disease Flashcards
Takayasu epidemiology
1) female 6-8x more
2) 20-30’s
3) more in East Asians
4) 1 in 3000 Japanese
First Takayasu arteritis
18th century Morgagni
First AV anastamosis of ocular papilla in 21 yo woman with vision loss
1908 Mikito Takayasu
Genotype of Takayasu
1) HLA-Bw52 (44% of Japanese TA) - Dw12 even worse
2) HLA-A, -D, -DR region mutations
Immune disease associated with TA
1) RA
2) SLE
3) IBD
4) glomerulonephritis
Other immune association in TA
1) Hsp60 cell-mediated mechanism
2) antibody-mediated mechanism
3) sex hormone relationship
Pathology of TA
1) panarteritis of all walls
2) skip lesions
3) inflammation start in vasa vasorum
4) SMC and fibroblast invade intima
How does aneurysms occur on a cellular level in TA
Destruction of wall occurs before fibrosis sets in
Three phases of TA
1) inflammatory period: constitutional symptoms
2) vessel inflammation: vessel pain, tenderness, carotodynia
3) burn-out phase: vessel fibrosis and aneurysm degeneration
Not all go through the same phases
% of patients with TA that are asymptomatic at presentation
10%
% of patients with TA that never develop constitutional symptoms
57%
Delay in diagnosis of TA
1 year
Symptoms of TA
1) carotid bruit (NA/Italy)
2) absent pulses (Mexico)
3) HTN (India) 33-60%
4) Carotodynia 32%
5) TIA/stroke 5-20%
6) visual disturbances 30%
7) diminished UE pulse 53-98%
8) UE claudication 62%
9) cardiac involvement
10) renal artery stenosis 20-50%
11) visceral 3-31%
12) LE ischemia 24-32%
13) raynaud 8-14%
14) skin changes 8-25%
Cardiac involvement of TA and manifestations
1) coronary 6-16%
2) aortic regurg 12-24% due to dilated root
3) mitral regurg 3-11%
4) pulmonary artery
HTN in TA key points
1) bilateral stenosis of subclavian makes measurement harder
2) mid aortic syndrome 16% of TA
Causes of mid aortic syndrome
1) TA
2) congenital hypoplasia
3) von Recklinghausen disease
4) FMD
5) tuberculosis
Aneurysm in TA frequency in named vessels
1) Abd aorta
2) subclavian artery
3) thoracic artery
Rate of aneurysm 7-71%
Rupture risk lower than other types of aneurysms
Cutaneous skin changes of TA
1) erythema nodosum
2) pyoderma gangrenosum
3) lupuslike malar flush
American college of rheumatology 1990 TA criteria
3/6
sen 90.5
spe 97.8
1) Age < 40
2) claudication of extremity
3) decreased brachial artery pulse
4) BP difference > 10 mmHg
5) bruit over subclavian artery or aorta
6) arteriogram abnormality
Sharma criteria TA 1996
MAJOR
1) left mid subclavian lesion
2) right mid subclavian lesion
3) symptoms > 1 month
MINOR
1) elevated ESR
2) carotid tenderness
3) HTN
4) aortic regurg
5) pulmonary
6) left CCA disease
7) distal brachiocephalic lesion
8) descending aortic lesion
9) abdominal aortic lesion
10) coronary lesion
- two major
-one major two minor
-4 minor
sen 96; spe 96
Biomarkers elevated in TA
1) ESR
2) CRP
3) IL6
4) RANTES (regulated upon activation normal T-cell expressed and presumably secreted)
5) MMP2, 3, 9
Skip vs contiguous lesions in TA
skip 16%
contiguous 81%
Types of TA classification by Ueno 1967
TYPE 1: confined to aortic arch and branches
TYPE 2: descending and abdominal aorta and branches
TYPE 3: combination of 1+2
TYPE 4: pulmonary artery involvement
1994 Tokyo international conference on takayasu in 6 patterns
TYPE 1: aortic arch branches
TYPE 2a: arch and branches
TYPE 2b: aortic arch, ranches and descending
TYPE 3: descending and abdominal aorta and branches
TYPE 4: abdominal aorta and branches
TYPE 5: entire aorta and branches
C: coronary
P: pulmonary
% of TA with self-limited disease
12-20%
Treatment of TA medication
Glucocorticoids 1mg/kg/day for 1-3 months
Success 20-100%
Others
1) methotrexate
2) cyclophosphamide
3) azathiaprine
4) mycophenolate mofetil
5) tocilizumab (anti IL6)
6) rituximab (B-cell depletion)
Relapse in TA
45-96%
Revascularization in TA 5 year patency during remission vs active
88% if done during remission
53% in active disease
Indication for surgery in TA
1) HTN in renal artery stenosis
2) lifestyle-limiting extremity ischemia
3) cerebral ischemia
4) > 70% symptomatic stenosis of cerebral vessel
5) moderate Gr II NYHA aortic regurg
6) cardiac ischemia
7) severe aortic coarctation
8) progressive aneurysmal enlargement
9) dissecting aneurysm
POBA patency for initial and 5 years for renal stenosis and mid aortic
renal 83%
5 year 33%
mid aortic 100%
5 year 33%
Coexistence of aortic regurg and coronary disease
45%
5 year primary and secondary patency after renal bypass in TA
79%
89%
Difference in TA vs atherosclerotic carotid disease
1) longer segment in TA
2) not ammenable to CEA
3) thrombosis not emboli
Results of open revascularization of TA mid aortic syndrome
1) 75% improvement in HTN
2) 50% no longer needing HTN meds
3) post-op event-free survival greatest in those who experience resolution of HTN
Most common cause of late mortality in TA
CHF
20 year survival of TA
74%
Anastomostic aneurysm after TA repair
only higher if for aneurysm
if for thrombotic then risk is similar to other indications
Poor maternal and fetal outcome indicators
1) HTN
2) aneurysmal disease
3) cardiac failure
4) extent of disease
