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Unusual Presentation of Takayasu Arteritis
Unusual Presentation of Takayasu Arteritis
Unusual Presentation of Takayasu Arteritis
Unusual Presentation of Takayasu Arteritis
Unusual Presentation of Takayasu Arteritis
Unusual Presentation of Takayasu Arteritis
Unusual Presentation of Takayasu Arteritis
Unusual Presentation of Takayasu Arteritis
Unusual Presentation of Takayasu Arteritis
Unusual Presentation of Takayasu Arteritis
Unusual Presentation of Takayasu Arteritis
Unusual Presentation of Takayasu Arteritis
Unusual Presentation of Takayasu Arteritis
Unusual Presentation of Takayasu Arteritis
Unusual Presentation of Takayasu Arteritis
Unusual Presentation of Takayasu Arteritis
Unusual Presentation of Takayasu Arteritis
Unusual Presentation of Takayasu Arteritis
Unusual Presentation of Takayasu Arteritis
Unusual Presentation of Takayasu Arteritis
Unusual Presentation of Takayasu Arteritis
Unusual Presentation of Takayasu Arteritis
Unusual Presentation of Takayasu Arteritis
Unusual Presentation of Takayasu Arteritis
Unusual Presentation of Takayasu Arteritis
Unusual Presentation of Takayasu Arteritis
Unusual Presentation of Takayasu Arteritis
Unusual Presentation of Takayasu Arteritis
Unusual Presentation of Takayasu Arteritis
Unusual Presentation of Takayasu Arteritis
Unusual Presentation of Takayasu Arteritis
Unusual Presentation of Takayasu Arteritis
Unusual Presentation of Takayasu Arteritis
Unusual Presentation of Takayasu Arteritis
Unusual Presentation of Takayasu Arteritis
Unusual Presentation of Takayasu Arteritis
Unusual Presentation of Takayasu Arteritis
Unusual Presentation of Takayasu Arteritis
Unusual Presentation of Takayasu Arteritis
Unusual Presentation of Takayasu Arteritis
Unusual Presentation of Takayasu Arteritis
Unusual Presentation of Takayasu Arteritis
Unusual Presentation of Takayasu Arteritis
Unusual Presentation of Takayasu Arteritis
Unusual Presentation of Takayasu Arteritis
Unusual Presentation of Takayasu Arteritis
Unusual Presentation of Takayasu Arteritis
Unusual Presentation of Takayasu Arteritis
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Unusual Presentation of Takayasu Arteritis

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  • 1. An Interesting case of Quadriparesis S.Karthikeyan., M4 Prof.P.Vijayaraghavan`s unit
  • 2. <ul><li>A 37 yr old female, admitted on 25/5/11 with </li></ul><ul><li>c/o difficulty in using both upper and </li></ul><ul><li>lower limbs – past 5 hrs </li></ul><ul><li>On elaborating: </li></ul><ul><li>Patient was apparently normal a few hrs before admission </li></ul><ul><li>- she had 3—4 episodes of vomiting ,non projectile, non bilious </li></ul><ul><li>- she went to bed normally and found herself after an hour to have difficulty in using both upper and lower limbs </li></ul>
  • 3. <ul><li>CONTD… </li></ul><ul><li>Weakness --- flaccid type </li></ul><ul><li>symmetrical </li></ul><ul><li>involved both UL&amp;LL equally </li></ul><ul><li>involved both proximal and distal </li></ul><ul><li> muscles equally </li></ul><ul><li>No h/o difficulty in lifting head from bed </li></ul><ul><li>No h/o difficulty in respiration </li></ul><ul><li>No history s/o sensory disturbances </li></ul><ul><li>No h/o bladder disturbances </li></ul>
  • 4. <ul><li>No h/o altered sensorium </li></ul><ul><li>No h/o headache ,blurring of vision diplopia </li></ul><ul><li>No h/o involuntary movements </li></ul><ul><li>No history s/o any cranial nerve involvement </li></ul><ul><li>No h/o neck pain or radiating pain down the limbs </li></ul><ul><li>h/o pain in distal &amp; proximal parts of both ULs - crampy, intermittent, occurs upon exercise, relieved by rest promptly </li></ul>
  • 5. <ul><li>No h/o change in colour of hand/fingers upon exercise or exposure to cold </li></ul><ul><li>but feels a sensation of cold upon exercise </li></ul><ul><li>No h/o joint pain, swelling,rashes </li></ul><ul><li>No h/o chest pain,palpitation,syncope </li></ul><ul><li>No h/o fever , weight loss or loss of appetite </li></ul><ul><li>No h/o trauma </li></ul><ul><li>No h/o recent vaccination,dog bite </li></ul>
  • 6. <ul><li>Past history: </li></ul><ul><li>No h/o similar complaints in past </li></ul><ul><li>No h/o HT/DM/CAD/BA/Epilepsy/TB </li></ul><ul><li>Personal history: </li></ul><ul><li>Not a smoker,alcoholic,tobacco chewer </li></ul><ul><li>menstrual periods– normal </li></ul><ul><li>Family history : </li></ul><ul><li>no h/o similar illness in family </li></ul>
  • 7. <ul><li>O/E: </li></ul><ul><li>Conscious,oriented </li></ul><ul><li>pallor+, </li></ul><ul><li>no icterus ,cyanosis,clubbing,pedal edema </li></ul><ul><li>no lymphadenopathy </li></ul><ul><li>well hydrated </li></ul><ul><li>JVP not elevated </li></ul>
  • 8. <ul><li>Pulse chart: </li></ul><ul><li>Pulse: 64/mt, regular rhythm normal volume,no specific character </li></ul><ul><li>B.P:160/100 mm hg in both lower limbs </li></ul>Artery Right Left Carotid + + Radial -- -- Brachial -- -- Femoral + + Popliteal + + Dorsalis pedis + +
  • 9. <ul><li>CVS: S1S2 + , no murmers </li></ul><ul><li>RS: NVBS </li></ul><ul><li>ABDOMEN: soft, no organomegaly </li></ul><ul><li>CNS: conscious,oriented </li></ul><ul><li>speech: normal </li></ul><ul><li>memory : intact </li></ul><ul><li>No e/o any cranial nerve involvement </li></ul>
  • 10. <ul><li>Motor system: </li></ul><ul><li>R L </li></ul><ul><li>Bulk UL normal normal </li></ul><ul><li>LL normal normal </li></ul><ul><li>Tone UL decreased decreased </li></ul><ul><li>LL decreased decreased </li></ul><ul><li>Power UL 2/5 2/5 </li></ul><ul><li>LL 2/5 2/5 </li></ul>
  • 11. Superficial reflexes R L corneal + + conjunctival + + gag + + palatal + + abdominal + + cremastric + + plantar flexor flexor
  • 12. DTRs R L biceps + + triceps + + supinator + + knee + + ankle + +
  • 13. <ul><li>Gait and co ordination : not tested </li></ul><ul><li>Sensory system:intact </li></ul><ul><li>Cerebellar function test : not tested </li></ul><ul><li>Spine &amp; cranium:normal </li></ul><ul><li>No e/o meningeal signs </li></ul>
  • 14. <ul><li>Problems : </li></ul><ul><li>Acute onset flaccid quadriparesis </li></ul><ul><li>Non palpable upper limb pulses </li></ul><ul><li>Hypertension </li></ul>
  • 15. &nbsp;
  • 16. Investigations <ul><li>CBC RFT: </li></ul><ul><li>Hb:11.0 RBS:95 mg </li></ul><ul><li>Tc:8600 B.Urea:22 mg </li></ul><ul><li>DC:P68,L30,E2 Sr.creatinine:0.7 mg </li></ul><ul><li>RBC:3.7 Million Sr.electrolytes: </li></ul><ul><li>MCV:90 Na--137 </li></ul><ul><li>MCH:30 K – 1.9 </li></ul><ul><li>ESR:5/12 Cl – 108 </li></ul><ul><li>Mg- 2 </li></ul><ul><li>Ca – 10 </li></ul><ul><li>Urine routine: normal study </li></ul><ul><li>CPK --- 150 U/L </li></ul>
  • 17. <ul><li>ECG: 60/mt,NSR, Normal Axis, </li></ul><ul><li>prominent `U` waves,reduced `T` wave amplitude </li></ul><ul><li>CXR:Normal study </li></ul>
  • 18. INITIAL TREATMENT <ul><li>Oral fluids/feeds rich in potassium </li></ul><ul><li>Syrup KCL 15—30 ml hourly </li></ul><ul><li>T.Amlodipine 2.5 mg od </li></ul><ul><li>T.Rantac 150 mg bd </li></ul><ul><li>T.BCT 1 bd </li></ul><ul><li>ECG monitoring </li></ul><ul><li>Over the next few hours weakness improved gradually and pt become ambulant next day </li></ul>
  • 19. <ul><li>SERIAL Potassium values: </li></ul>DAY 25/5 26/5 28/5 30/5 Sr.K 1.9 3.2 3.2 3.4
  • 20. &nbsp;
  • 21. Urine analysis <ul><li>24 hr urine sample </li></ul><ul><li>30/5 4/6 </li></ul><ul><li>Na ---- 180 meq/d Na----170 </li></ul><ul><li>K ---- 150 meq/d K ---- 148 </li></ul><ul><li>Ca ---- 144 mg/d Ca ----150 </li></ul><ul><li>Cl ---- 138 meq/d Cl ---- 142 </li></ul>
  • 22. ABG <ul><li>METABOLIC ALKALOSIS WITH COMPENSATORY </li></ul><ul><li>RESPIRATORY ACIDOSIS WITH NORMAL ANION GAP </li></ul><ul><li>It was persistent when repeated thrice in a span of 10 days </li></ul>
  • 23. <ul><li>Hypokalemia </li></ul><ul><li>renal potassium wasting </li></ul><ul><li>metabolic alkalosis </li></ul><ul><li>hypertension </li></ul><ul><li>The DDs are 1)Mineralocorticoid excess </li></ul><ul><li>2)Liddle syndrome </li></ul>
  • 24. Next step… <ul><li>“ GOT STUCK” </li></ul><ul><li>Sr.Renin and Aldosterone not done </li></ul>
  • 25. IMAGING <ul><li>USG abdomen/KUB: Normal study </li></ul><ul><li>CT Abd: normal study </li></ul><ul><li>Renal artery doppler: </li></ul><ul><li>Resistive index </li></ul><ul><li>Normal : less 0.8 </li></ul><ul><li>Impression: distal stenosis of renal artery ? Segmental arteries of right kidney </li></ul>R L Upper pole 0.94 0.68 Mid pole 0.90 0.64 Lower pole 0.78 0.64
  • 26. Ct angiogram
  • 27. &nbsp;
  • 28. &nbsp;
  • 29. &nbsp;
  • 30. &nbsp;
  • 31. &nbsp;
  • 32. Report <ul><li>Ct angiogram:B/L thinning of subclavian artery with stenosis and tortuosity in its course </li></ul><ul><li>Diffuse aortic wall thickening </li></ul><ul><li>Normal appearing carotid , vertebral, abdominal aorta and proximal renal arteries </li></ul>
  • 33. <ul><li>“ Disease affecting aorta, its branches and possibly distal renal arteries “ </li></ul>
  • 34. POSSIBILITIES ARE…………. DISEASE TYPE ENTITIES RHEUMATIC GIANT CELL arteritis,Cogan syndrome,Relapsing polychondritis,Ankylosing spondylitis,Rheumatoid arthritis,SLE,buerger`s disease INFECTIOUS Syphilis,TB OTHERS Atherosclerosis,ergotism,radiation induced damage,sarcoidosis,marfan syndrome,Ehler Danlos syndrome ,congenital co arctation of aorta
  • 35. <ul><li>Other investigations: </li></ul><ul><li>Mantoux : negative </li></ul><ul><li>VDRL:negative </li></ul><ul><li>ANA: negative </li></ul><ul><li>RA Factor:negative </li></ul><ul><li>CRP:5mg/L </li></ul><ul><li>Lipid profile: normal limits </li></ul><ul><li>Sr .T3,T4,TSH--wnl </li></ul><ul><li>CT chest: normal study </li></ul><ul><li>ECHO: normal study </li></ul>
  • 36. &nbsp;
  • 37. Queries??????? <ul><li>Why vessel wall biopsy not done in this case? </li></ul><ul><li>CT –Angio doesn show e/o renal artery stenosis.. How to explain? </li></ul>
  • 38. Takayasu&apos;s arteritis <ul><li>Takayasu&apos;s arteritis  is also known as &amp;quot;aortic arch syndrome&amp;quot;, &amp;quot;nonspecific aortoarteritis&amp;quot; and the &amp;quot;pulseless disease”. </li></ul><ul><li>It is a form of large vessel and medium granulomatous vasculitis with massive intimal fibrosis and vascular narrowing. </li></ul><ul><li>Incidence:0.2—2.5/million/yr </li></ul><ul><li>It affects young or middle-aged women M:F 8:1. </li></ul><ul><li>ASIANS commonly affected </li></ul><ul><li>It mainly affects the aorta (the main blood vessel leaving the heart) and its branches, as well as the pulmonary arteries. </li></ul>
  • 39. Pathogenesis <ul><li>TA is characterized by granulomatous inflammation of the aorta and its major branches, leading to stenosis, thrombosis, and aneurysm formation. </li></ul><ul><li>The lesions of TA are segmental with a patchy distribution. </li></ul><ul><li>Mononuclear infiltration of the adventitia occurs early in the course of the disease, with cuffing of the vasa vasorum. </li></ul><ul><li>There is no inflammatory infiltrate of the intima; changes are thought to be reactive to inflammation in the media and adventitia. </li></ul>
  • 40. Symptoms <ul><li>Systemic symptoms in Takayasu arteritis (TA) include the following: </li></ul><ul><li>Fever, night sweats </li></ul><ul><li>Fatigue </li></ul><ul><li>Weight loss </li></ul><ul><li>Myalgia and/or arthralgia and/or arthritis </li></ul><ul><li>Skin rash (eg, erythema nodosum, pyoderma gangrenosum) </li></ul><ul><li>Headaches and/or dizziness and/or syncope </li></ul><ul><li>Congestive heart failure, palpitations, angina </li></ul><ul><li>Hypertension (may be paroxysmal) </li></ul>
  • 41. 1990 Criteria of American College of Rheumatology for the Classification of Takayasu Arteritis Criteria Definition Age at disease onset in year  Development of symptoms or findings related to Takayasu arteritis at age &lt;40 years.  Claudication of extremities  Development and worsening of fatigue and discomfort in muscles of one or more extremity while in use, especially the upper extremities.  Decreased brachial artery pulse Decreased pulsation of one or both the brachial arteries BP difference &gt;10mmHg Difference of &gt;10mmHg in systolic blood pressure between arms  Bruit over subclavian arteries or aorta Bruit audible on auscultation over one or both subclavian arteries or abdominal aorta Arteriogram abnormality  Arteriographic narrowing or occlusion of the entire aorta, its primary branches, or large arteries in the proximal uppper or lower extremities, not due arteriosclerosis, fibro-muscular dysplasia, or similar causes: changes usually focal or segmental  For purposes of classification, a patient shall be said to have Takayasu&apos;s arteritis if at least three of these six criteria are present. The presence of any three or more criteria yields a sensitivity of 90.5% and a specificity of 97.8%. BP = blood pressure (systolic) difference between arms
  • 42. TYPES TYPES 1 Aortic arch and branches 2 Abdominal aorta 3 Both arch and abdominal aorta 4 Pulmonary artery
  • 43. Treatment <ul><li>Daily high-dose corticosteroid administration is the mainstay of initial therapy for active disease. </li></ul><ul><li>Prednisone at 1-2 mg/kg/d for 4-6 weeks. </li></ul><ul><li>Patients not responding to corticosteroids or who relapse during corticosteroid taper require an additional agent. </li></ul><ul><li>Regimens including weekly methotrexate or daily or monthly intravenous cyclophosphamide . </li></ul><ul><li>Cyclosporine may be an alternative therapy offering lower ovarian toxicity than cyclophosphamide. </li></ul><ul><li>Mycophenolate mofetil may be useful to treat individuals with glucocorticoid-resistant disease. </li></ul><ul><li>Infliximab has been used in children with Takayasu arteritis. </li></ul><ul><li>Anecdotal reports of matrix metalloproteinase inhibition using minocycline propose that this may be a useful adjunctive therapy. </li></ul>
  • 44. <ul><li>Surgical therapy: </li></ul><ul><li>Bypass surgeries for stenotic lesions </li></ul><ul><li>PTCA for stenotic renal arteries </li></ul><ul><li>Aortic valve replacement for AR </li></ul>
  • 45. PROGNOSIS <ul><li>20% have self limited disease </li></ul><ul><li>Rest have relapsing and remitting disease </li></ul><ul><li>5yr survival rate is 92.5% </li></ul><ul><li>Death is due to cardiac and renal failure </li></ul>
  • 46. Carry home points…….. <ul><li>TA is a vasculitis affecting large and medium sized arteries </li></ul><ul><li>Young females more commonly affected </li></ul><ul><li>Angiography is an important tool in diagnosis </li></ul><ul><li>Vessel biopsy is not mandatory </li></ul><ul><li>Corticosteroids are the mainstay in treatment </li></ul><ul><li>Infliximab – a newer modality in treatment </li></ul><ul><li>Cardiac and renal failure are common causes of death </li></ul>
  • 47. References <ul><li>Harrison`s Principles of Internal Medicine </li></ul><ul><li>17 edition </li></ul><ul><li>Kelly`s Text book of Rheumatology </li></ul><ul><li>eMedicine </li></ul><ul><li>Rheumatology case reports </li></ul>
  • 48. <ul><li>Thank you </li></ul>

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