Takayasu arteritis (case presentation)

Salman Saleem
FCPS-II trainee

Name: aaa
Age: 22 years
Gender: female
Marital Status: Unmarried
Resident of lahore
Date of presentation: 22nd May 2014

Presented with chief complaint of
Left upper limb pain-----------1month

My patient had pain in left upper arm, most
sever in left forearm and hand which was
Aching in nature, present most of the time
and aggravated by doing any kind of work.
There was associated numbness and
coldness of the left hand.
There is no history of any color changes
on exposure to cold. There is no H/O skin
rash or fever

 There is H/O generalized body aches and
pains, sometimes left shoulder pains but no
systemic joint pains, stiffness, redness or
swelling.
 There is no HO lower limb pain or
claudication. No Chest pain or SOB
 No HO vertigo, dizziness, blackouts or
headache
 No HO cough sputum, hemoptysis, burning
micturtion, hematuria, pyuria
 No GIT disturbance
 Normal periods.

 She had Hx of fever 07 months back.
 The fever continued for 01 month. It was mostly low
grade, intermittent. fever was associated body aches
and join pains involving both small and large joints.
There was no joint swelling or stiffness
 There was also dry cough and exercise intolerance.
There was no HO hematuria or burning. No GIT
symptoms.
 No hx Skin rash
 She remained under treatment from various physicians
and got worked up to find out the cause of fever but no
conclusive diagnosis could be made. The fever finally
subsided after 01 month

 Hb: 11.2
 TLC: 6.2
 PLT: 335000
 MCV: 86
 Urea: 17
 Creatinine: 0.5
 Uric acid: 3.1
 Bil: 0.4
 SGPT: 23
 Alk Po: 81
 Calcium: 10.0
 25 OH vit D3: 37.32
 Total protein: 7.8
 Alb: 4.6
 Globulin: 3.2
 ESR: 70
 CRP: < 6
 ANA: -ve
 RA Factor: -ve
 Anti CCP: -ve
 Transthoracic Echo:
Normal Valves normal systolic
and diastolic dimensions
and EF of 55%

Family history: no history IHD,DM or
dyslipidemia any similar illness in family
Personal Hx: She is staff nurse by
profession
There is no HO, smoking, addiction or drug
dependance
Menstrual History: noramal

 Young female of average height and built looks
anxious
 BP: 110/70 in rt arm and was not recordable in left
arm
 PULSE: 90 b/min
 ABSENT LEFT RADIAL. Left brachial but ulnar artery
was very feeble. There was no change in pulse with
change of position of left UL or neck
 All other pulses were normal. no radiofemoral delay
 R/rate: 18/min
 Temp: 98.6F

 There was no clubbing, cyanosis or edema
 Chest: Normal vesicular breathing
Abd: Soft non tender no palpable
visceromegaly
 CVS: S1 and S2 audible with no murmur
CNS: Normal examination

1)Vasculitis:
 Large vessel:Takayasu’s arteritis , Giant cell arteritis,
2)Atherosclerosis
3)Buerger’s disease
4)Thoracic outlet syndrome
5)Coarctation of Aorta
(Medium vessel vasulitis: Polyarterititis nodosa,
kawasaki’s disease
Small vessel: wegner’s granulomatosis , rheumatoid arthritis,)
were also in differentials

 Giant cell arteritis occurs in elderly with headache
and jaw claudication
 Thoracic outlet syndrome: its pain is aggravated with movement in vascular type
thoracic outlet syndrom
 Coarctation of Aorta: Pt has no radiofemoral delay and hypertention
 Athersclerosis: donot involve subclavian artery as individual . No family history
of dyslipidemia
 Buerger’s disease ocuurs in middle aged smoker male and effects lower limb
 PAN associated with abdominal pain, rashes, hematuria and peripheral
neuropathy
 Kawasaki disease occurs in childhood pts have eye symptoms,
lymphadenopathy and cutaneous lesions
 Wegner’s Granulomatosis: is associted with upper respiratory symptoms and
hemoptysis
 Rheumatoid arthritis: ho morning stiffness and multi organ involvement

CBC,ESR,ANA, RA factor, ENA, C-ANCA,
P-ANCA,
LFTS,RFTs,Lipid porfile
ECG
CXR
Arterail Doppler
CT Peripheral Angiogram was planned

 Hb: 10.2
 Plt: 453000
 Tlc: 7800
 Mcv: 80.6
 ESR: 58
 CRP: <6
 Urea: 17
 Creatinine: 0.5
 S. Bil: 0.4
 SGPT: 23
 ALT: 32
 Alk Phosp:81
 Calcium: 8.9
 Phosph: 3.9
 Vit D3 : 37
 Albumin: 4.6
 Globulin: 3.2

 ANA: -ve
 RA Factor:-ve
 Anti CCP:-ve
 HBsAg:-ve
 Anti HCV:-ve
 Arterial doppler of left
upper Limb Shows
stenosis of the left
subclavian artery with
diminished flow
beyond

Investigation Oct-
Nov:2013
May-june
2014
Echo: Dec: 2013:
Normal Valves and NORmal
dimensions and Normal
systolic Function
Ultra Sound Doppler 6th May
2014:
Show stenosed left subclavian
artery with diminished flow
beyond and normal flow in rt
arm
CBC Hb:11.2
TLC:6.2
PLT:325
MCV:86
Hb:10.2
TLC:7.8
PLT:453
MCV:80
ESR
CRP
70
<6
58
<6
Antibody ANA: -ve
RA factor: -ve
AntiCCP: -ve
Others HBsAg and
ANTIHCV:-ve
Ca:10.0
LFT & RFT:
WNL
LFTs and
RFTs :
WNL

1) Age younger than 40yrs at disease onset
2) Claudication of the extremities
3) Decreased pulsation of one or both brachial arteries
4) Difference of at least 10 mm Hg in systolic blood
pressure between arms
5) Bruit over 1 or both subclavian arteries or the
abdominal aorta
6) Arteriographic narrowing or occlusion of the entire
aorta, its primary branches, or large arteries in the
upper or lower extremities that is not due to
arteriosclerosis, fibromuscular dysplasia, or other
causes
The presence of any 3 or more criteria yields a
sensitivity of 90.5% and a specificity of 97.8%.[20]

 First to treat as medically as shown by ESR &
symptoms pt was in active stage and then to
do intervention of left subclavian artery
 So Pt was prescribed deltaacortil 30 mg per
along with Methotrexate 10 mg weakly
 Pts symptoms improved
ESR dec: 70  08
 Methotrexate was stopped due to
hepatotoxicity
 And finally stenting of left subclavian artery
was done

 Is inflammatory arteritis of unknown origin involving
medium and large size arteries.
 Granulamtous inflammation of large arteries and
medium sized arteies
 Circulating antibodies plays important role
 There may be stenosis (75%), occlusion or
aneurysm of the arteries causing ischemic
symptoms
 May involve single branch of aorta or all arteries
arising from aorta

 Incidence of 2.6 cases per 1 million
 It is more prevalent in japan and other Asian countries
 More prevalent in female (8:1 ratio)
 Remitting and relapsing very prolonged course that
extend over years
 Three stages:
1) Active inflammatory phase (non constitutional
symptom)
2) Vascular inflammatory stage( vascular stage)
3) Burnt out stage: (vessels become fibrosed )

Type I - Branches of the aortic arch
Type IIa - Ascending aorta, aortic arch, and its
branches
Type IIb –Ascending, Arch plus thoracic
descending aorta
Type III - Thoracic descending aorta,
abdominal aorta, renal arteries, or a
combination
Type IV - Abdominal aorta, renal arteries, or
both
Type V - Entire aorta and its branches

 Signs
 Non specific like fever,
rash, joint pain,weight
loss
 Limb claudication
 Headache
 Visual disturbance
 Reynaud’s phenomenon
 Symptoms
 Hypertension
 Pressure difference b/w
two arms
 Bruit
 Signs of Aortic
regurgitation
 Signs of Bi ventricular
failure

1) Age of 40 years or younger at disease onset
2) Claudication of the extremities
3) Decreased pulsation of 1 or both brachial arteries
4) Difference of at least 10 mm Hg in systolic blood
pressure between arms
5) Bruit over 1 or both subclavian arteries or the
abdominal aorta
6) Arteriographic narrowing or occlusion of the entire
aorta, its primary branches, or large arteries in the
upper or lower extremities that is not due to
arteriosclerosis, fibromuscular dysplasia, or other
causes
The presence of any 3 or more criteria yields a
sensitivity of 90.5% and a specificity of 97.8%.[20]

Increase in Acute phase reactant like ESR
& CRP
Increased APR may show active disease
but may be normal
Patient may shows normocytic anemia and
thrombocytosis
All antibodies like ANA,ANCA, CCP will be
negative

 Echocardiogram:
may show valvular
abnormality ( AR) or bi ventricular failure due to
myocarditits
 CT angiogram:
it is non invasive and detects early
disease
 MR angiogram:
detects early disease provide
detailed information
 Angiography :
of affected artery is gold standard

 LIMB ISCHEMIA
SUBCLAVIAN STEAL SYNDROME
CVA
 Renovascular Hypertension when involve
renal artery
 Thromboembolic phenomenon
 Retinpathy
 Bi-ventricular failure
Valvular abnormality most common is Aortic
regurgitation
 Complication related to prolonged steroids
use

Assessing disease activity may be
beneficial as it respond to steroid.
presence of any of the following 2 out of 4
suggest active disease
1)Sytsemic features like fever and
arthralgias
2)inc: ESR
3)Features of ischemia like claudication
4)Typical angiographic features

 Steroids like predisnolone 1mg/kg
 Steroids usually benefits patients with active
disease donot effect burnt out fibrosed
vessels
 ½ of all pts on steroids do relapses after
stoping
 Adding immunosuppressive to non
responders
 Cyclophosphamide daily & Methotrexate
weekly can be used to reduce dose of
steroids
 Anti TNF therapy

 Literature had showed better outcome with
PTA because it can be reperformed
There can be restenosis of stented vessel
which is usually greater than PTA
performed in the Atherosclerosis lesions
However surgical grafting can achieve
better outcome with greater risk

Takayasu arteritis (case presentation)

Takayasu arteritis (case presentation)

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