This document describes the case of a 22-year-old female who presented with 1 month of left upper limb pain. On examination, she had absent left radial pulse and diminished left brachial and ulnar pulses. Tests showed elevated ESR and stenosis of the left subclavian artery on arterial Doppler. She was diagnosed with Takayasu's arteritis based on meeting criteria including age less than 40 and arterial narrowing. She was treated with steroids and methotrexate, which improved her symptoms, and later underwent stenting of the left subclavian artery. Takayasu's arteritis is an inflammatory condition involving medium and large arteries that commonly affects young females and can cause limb ischemia.
4. 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
5. 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.
6. 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
7. 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%
8. 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
9. 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
10. 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
11. 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
12. 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
13. CBC,ESR,ANA, RA factor, ENA, C-ANCA,
P-ANCA,
LFTS,RFTs,Lipid porfile
ECG
CXR
Arterail Doppler
CT Peripheral Angiogram was planned
15. 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
16. 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
20. 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]
21. 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
23. 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
24. 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 )
25. 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
26. 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
27. 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]
28. 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
29. 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
30. 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
31.
32. 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
33. 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
34. 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
Editor's Notes
Giant cell arteritis usually affect older age> 60 and causes headache and joint claudication
Polyarteritis nodosa causes abdominal pain and rashes and peripheral neuropathy and involve renal artery as well
Kawasaki disease is usually present in early childhood and may cause myocardial infarction in early childhood and involves conjunctiva in 90%
wegner’s granulomatosis causes upper respiratory symptoms and hemoptysis, there was no hx of morning stiffness and symmetrical joint pain and anti ccp was negative so this is also excluded buerger’s disease occurs in middle aged smoker male and invove claudication of lower limb
Thoracic outlet syndrom rarely causes artrial insufficiency by compressing left subclavian artery and pain is aggravated by overhead movement of arm which is not the case
Following investigations were advised based on history and Examination
She has anemia with normal MCV possibility of anemia of Chronic disease her ESR which was raised in past also raised here
To summarize the patient she is 22 yrs old female with history of left upperlimb claudication for one month with past history PUO that settled spontaneously
She was diagnosed as a case of takayasu arteritis on following ACR criteria
Pt had 5 criteria positive out of 6
Mycobacterial tuberculosis and streptocoal infection have been reported as a possible culprits
Aortic regurgitation most commonly caused by aortic aneurysm involving
Whenever there is ipsilateral subclavian artery occlusion the there is retrograde flow of blood from vertebral to subclavian artery in order to provide blood thru dilated collateral vessels and results in ischemic symptoms due to brain