Chapter 139 - Takayasu Disease

Question 1

Takayasu epidemiology

Answer 1

1) female 6-8x more
2) 20-30’s
3) more in East Asians
4) 1 in 3000 Japanese

Question 2

First Takayasu arteritis

Answer 2

18th century Morgagni

Question 3

First AV anastamosis of ocular papilla in 21 yo woman with vision loss

Answer 3

1908 Mikito Takayasu

Question 4

Genotype of Takayasu

Answer 4

1) HLA-Bw52 (44% of Japanese TA) - Dw12 even worse

2) HLA-A, -D, -DR region mutations

Question 5

Immune disease associated with TA

Answer 5

1) RA
2) SLE
3) IBD
4) glomerulonephritis

Question 6

Other immune association in TA

Answer 6

1) Hsp60 cell-mediated mechanism
2) antibody-mediated mechanism
3) sex hormone relationship

Question 7

Pathology of TA

Answer 7

1) panarteritis of all walls
2) skip lesions
3) inflammation start in vasa vasorum
4) SMC and fibroblast invade intima

Question 8

How does aneurysms occur on a cellular level in TA

Answer 8

Destruction of wall occurs before fibrosis sets in

Question 9

Three phases of TA

Answer 9

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

Question 10

% of patients with TA that are asymptomatic at presentation

Answer 10

10%

Question 11

% of patients with TA that never develop constitutional symptoms

Answer 11

57%

Question 12

Delay in diagnosis of TA

Answer 12

1 year

Question 13

Symptoms of TA

Answer 13

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%

Question 14

Cardiac involvement of TA and manifestations

Answer 14

1) coronary 6-16%
2) aortic regurg 12-24% due to dilated root
3) mitral regurg 3-11%
4) pulmonary artery

Question 15

HTN in TA key points

Answer 15

1) bilateral stenosis of subclavian makes measurement harder

2) mid aortic syndrome 16% of TA

Question 16

Causes of mid aortic syndrome

Answer 16

1) TA
2) congenital hypoplasia
3) von Recklinghausen disease
4) FMD
5) tuberculosis

Question 17

Aneurysm in TA frequency in named vessels

Answer 17

1) Abd aorta
2) subclavian artery
3) thoracic artery

Rate of aneurysm 7-71%
Rupture risk lower than other types of aneurysms

Question 18

Cutaneous skin changes of TA

Answer 18

1) erythema nodosum
2) pyoderma gangrenosum
3) lupuslike malar flush

Question 19

American college of rheumatology 1990 TA criteria

Answer 19

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

Question 20

Sharma criteria TA 1996

Answer 20

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

Question 21

Biomarkers elevated in TA

Answer 21

1) ESR
2) CRP
3) IL6
4) RANTES (regulated upon activation normal T-cell expressed and presumably secreted)
5) MMP2, 3, 9

Question 22

Skip vs contiguous lesions in TA

Answer 22

skip 16%

contiguous 81%

Question 23

Types of TA classification by Ueno 1967

Answer 23

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

Question 24

1994 Tokyo international conference on takayasu in 6 patterns

Answer 24

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

Question 25

% of TA with self-limited disease

Answer 25

12-20%

Question 26

Treatment of TA medication

Answer 26

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)

Question 27

Relapse in TA

Answer 27

45-96%

Question 28

Revascularization in TA 5 year patency during remission vs active

Answer 28

88% if done during remission | 53% in active disease

Question 29

Indication for surgery in TA

Answer 29

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

Question 30

POBA patency for initial and 5 years for renal stenosis and mid aortic

Answer 30

renal 83% 5 year 33% mid aortic 100% 5 year 33%

Question 31

Coexistence of aortic regurg and coronary disease

Answer 31

45%

Question 32

5 year primary and secondary patency after renal bypass in TA

Answer 32

79% | 89%

Question 33

Difference in TA vs atherosclerotic carotid disease

Answer 33

1) longer segment in TA 2) not ammenable to CEA 3) thrombosis not emboli

Question 34

Results of open revascularization of TA mid aortic syndrome

Answer 34

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

Question 35

Most common cause of late mortality in TA

Answer 35

CHF

Question 36

20 year survival of TA

Answer 36

74%

Question 37

Anastomostic aneurysm after TA repair

Answer 37

only higher if for aneurysm | if for thrombotic then risk is similar to other indications

Question 38

Poor maternal and fetal outcome indicators

Answer 38

1) HTN 2) aneurysmal disease 3) cardiac failure 4) extent of disease