HISTORY A 36 year old female admitted with H/o pain in the Rt upper limb & Giddiness while working with the Rt upper limb for the past 6 months. She has right eye pain also while working with the upper limbs. No H/o fever, chest pain, dysponea, cough Bowel & Bladder habits normal

PAST HISTORY : No H/o DM / HT / PT / BA / IHD / Epilepsy PERSONAL & FAMILY HISTORY : Nil significant GENERAL EXAMINATION : Conscious, oriented, afebrile Pallor + No clubbing / cyanosis / PE / Jaun / GLA /elev.JVP

VITAL SIGNS - PULSE Arteries Rt Side Lt Side Superficial temporal + + CCA + thrill +, bruit + + Thrill +, bruit + Brachial feeble Radial feeble Femoral, PA, DPA, PTA + +

Blood Pressure: Limb Rt side Lt side UL 60/0 70/0 LL 210/0 220/0 RESPIRATORY RATE & TEMP: Normal

SYSTEMIC EXAMINATION: CVS : S1, S2 +, Hyperdynamic impulse EDM + in AA RS : NVBS +, No added sounds ABD : Soft, No mass palpable CNS : NFND

NECK: A 3 * 3 cm sized swelling palpable in the Lt carotid triangle, expansile impulse on palpation +, soft, smooth surface. ? ANEURYSM OF CAROTID ARTERY PROVISIONAL DIAGNOSIS AORTOARTERITIS / ?Carotid Aneurysm

INVESTIGATIONS Hb % : 10.4 gms TC : 9500 DC : P72 L26 E2 ESR : 10/25 PCV : 26 Urine R/E: Normal RBS : 119 mgs Bl.Urea: 16 mg S.Crea : 0.6 mg

S.Electrolytes: Na+ : 126 meq/L K+ : 2.8 meq/L LFT: STB: 0.9 mg SGOT: 19 IU/L SGPT : 18 IU/L SAP : 65 IU/L T.Protein : 7.5 gms Albumin : 4.8 gms ECG : LVH CXR : Aortic knuckle prominent &calcified Cardiomegaly +

ASO : Neg CRP : Neg RF : Neg ANA : Neg Mantoux test : Neg HIV : Neg VDRL : Neg Anti- HCV : Neg HBsAg :Neg

USG ABD: Prominent supra renal aorta ?Aneurysm, SMA prominent. Echo : AR (mod) No AS TR (mod) MV (normal) Total Cholesterol: 161 mgs Thyroid Function Tests: Normal Fundus : Normal

SURGICAL, RHEUMATOLOGICAL, CARDIAC OPINION : ? AortoArteritis VASCULAR SURGEON OPINION: ?Aorto Arteritis / ? Carotid Aneurysm, Suggested Doppler study Doppler Study of Vascular System : 1.Plaque in Rt CCA causing 59 % stenosis of CCA.Due to the stenosis there is no detectable diastolic flow in Rt ICA & Rt ECA

2. Diseased LCCA indicated by hyperechoiec and irregular intima, no detectable diastolic flow in Lt CCA indicating occlusion prior to LCCA origin. ? In Arch of Aorta 3. Due to the plaque highly pulsatile flow with nil diastolic flow noted in the Lt carotid bulb, Lt ICA & Lt ECA 4.Lt vertebral artery shows no diastolic flow but normal systolic velocity -? Arterial narrowing in ipsilateral SCA

5.Normal study of Renal arteries, infrarenal aorta, both lower limb arterial system. In these normal flow & velocity noted.

In our case……. There is claudication of upper extremities Aneurysm of carotid artery Aortic Regurgitation Feeble upper extremities pulses Typical Doppler study of arterial system …..

FINAL DIAGNOSIS AORTOARTERITIS/ AORTIC REGURGITATION

AORTOARTERITIS AA is a pan-arteritis involving all the three layers causing extensive intimal proliferation, inflammation of media & adventitia followed by marked fibrous scarring. Involves aorta & its major branches, pulmonary artery & its branches TYPES: TYPE I : Inflammatory process is localised to arch of aorta & its branches

TYPE II: Lesions involve the thoraco- abdominal aorta & its branches without the involvement of arch TYPE III: Combined Type I & Type II TYPE IV: Pulmonary involvement in addition to features of Type I,II or III TYPE V: Involvement of coronary arteries

COMMON PRESENTATIONS: 1.Unequal pulse 2.Hypertension(due to renal artery stenosis) 3.Renal failure 4.Heart failure(HT /Valvular lesions) 5.AR ( Root dilation / valvular) 6..Intermittent claudication 7.Aneurysmal AA( high incidence of AR,HT, Elevated ESR) 7.Others( CNS,Skin,eye changes)

Clinical Diagnosis of Aorto- Arteritis : I. One Obligatory criteria: < 40 yrs II. Two major criteria : Lt & Rt mid subclavian artery lesions III. Nine minor criteria : 1.High ESR 2.CCA tenderness 3. hypertension 4.Aortic Regurgitation 5.lesions of (pulmonary artery,Lt mid CCA, distal BCT, thoracic aorta & abdominal aorta High Probability of AA: OC + Two/one Major + >=2 / >=4 minor criteria

American College of Rheumatological criteria: 1. Age of onset of disease 10 mmHg bt arms 5.bruit over subclavian artery or aorta 6.arteriogram abnormality 3 of these 6 criteria suggests diagnosis of Aorto-Arteritis

TREATMENT Glucocorticoids Cytotoxic drugs (cyclophosphamide, azathioprine, methotrexate) Antihypertensives &decongestive therapy Percutaneous Transluminal angioplasty Surgery