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 disturbanceNormal 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

Takayasu’s Arteritis

1) Age younger than 40yrs at disease onset2) Claudication of the extremities3) Decreased pulsation of one or both brachial arteries4) Difference of at least 10 mm Hg in systolic blood

pressure between arms5) Bruit over 1 or both subclavian arteries or the

abdominal aorta6) 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 08Methotrexate was stopped due to

hepatotoxicityAnd finally stenting of left subclavian artery

was done

Diagnosis

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 archType IIa - Ascending aorta, aortic arch, and its

branchesType IIb –Ascending, Arch plus thoracic

descending aortaType 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,weightloss

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 onset2) Claudication of the extremities3) Decreased pulsation of 1 or both brachial arteries4) Difference of at least 10 mm Hg in systolic blood

pressure between arms5) Bruit over 1 or both subclavian arteries or the

abdominal aorta6) 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 ISCHEMIASUBCLAVIAN STEAL SYNDROMECVARenovascular Hypertension when involve

renal arteryThromboembolic phenomenonRetinpathyBi-ventricular failure Valvular abnormality most common is Aortic

regurgitationComplication 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: ESR3)Features of ischemia like claudication

4)Typical angiographic features

Steroids like predisnolone 1mg/kgSteroids usually benefits patients with active

disease donot effect burnt out fibrosedvessels

½ of all pts on steroids do relapses after stoping

Adding immunosuppressive to non responders

Cyclophosphamide daily & Methotrexateweekly 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