Sir, We would like to share our experience on a rare presentation of Takayasu arteritis (TA). A 32 year old male presented with chest pain from 3 days worsening on exertion and associated with shortness of breath. He is a chronic smoker and has a history of hyperlipidaemia. His examination was significant for diminished pulse in left upper limb and BP in the right upper limb was 150/90 mm of Hg. On auscultation cardiovascular and respiratory systems were unremarkable. His ECG showed diffuse ischemia (ST depression in infero-lateral leads). His cardiac enzymes (Troponin-T and CPK-MB) were negative. His ESR was high at 66mm/1st hr and C-reactive protein was 102mg/dl.2D echo revealed no regional wall motion abnormalities and ejection fraction of 63%. He was treated with antiplatelet therapy, heparin, nitroglycerin, Oxygen, ACE inhibitor and beta blocker. His coronary angiogram showed a normal LMCA, a diffusely diseased LAD-type III vessel; non-dominant LCX with two OMs all diffusely diseased having a beaded appearance (Figure-1). Aortitis was also distinctly evident from the irregular contour of the aorta (Figure-2). Diagnosed of TA, he was conservatively treated with steroids and anti-anginal medications and on follow up after 2 months he was significantly pain and symptom free along with normalized ESR and CRP. Coronary involvement in Takayasu arteritis (also called as Morterell syndrome or occlusive thromboaortopathy) is reported to be 9% to 11%.2 Pathologically coronary lesions may be classified as type 1- stenosis or occlusion of the coronary ostia and the proximal segments of the coronary arteries; type 2- diffuse or focal coronary arteritis, with skip lesions; type 3- coronary aneurysm and while all three may be seen in TA, type1 is more common.3 The extension of inflammation, which induces intimal proliferation and fibrous contraction in the ascending aorta around the coronary ostia is the proposed mechanism. Enhanced atherosclerosis accelerates the jeopardy of the vasculitis process. The biopsy might show intimal hyperplasia and a transmural lymphoplasmacytic infiltrate. Tunica media may be disorganized/ replaced by collagen and may feature patchy necrosis. Distorted and fragmented elastic fibers may predispose to dissection. The late “burned out” stage is marked by fibrous scarring, leading to intimal and adventitial thickening with dystrophic calcification. Steroids may achieve a 40% to 60% remission rate and about 40% of steroid-resistant patients respond with the addition of cytotoxic agents (20% are resistant to any kind of treatment).4 Apart from stenting, CABG, patch angioplasty of the LMCA ostium (ostioplasty using autologous pericardium/ saphenous vein graft/ internal thoracic artery), and transaortic coronary ostial endarterectomy and hybrid procedures (on porcelain aorta) are the available surgical options (to be avoided during the active stage of inflammation unless complicated by unstable angina).5 In a case of TA in a young female, an arterial Y graft was used with good results (probably from longer term graft patency).2 TA is an entity of substantial morbidity especially with coronary involvement and prompt immunosuppression and interventions (if necessary) can be life-saving.
Loading....