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DIAGNOSTIC DILEMMA • PRESENTATION • A 39-year-old Haitian woman arrived in the emergency department for evaluation of a severe headache and shortness of breath. She had been experiencing chest pain, both at rest and with exertion for several months. • Episodes lasted for 10-15 minutes and were substernal , very severe and squeezing in nature and were intermittently associated with dyspnea and nausea. The patient had been limiting her physical activity because she was afraid of precipitating chest discomfort. • A review of systems was otherwise notable for frequent headaches, a 5-10 pound unintentional weight gain, fatigue, and orthopnea. Exertion produced increased fatigue, dyspnea, and bilateral leg pain and “heaviness”. She denied lower-extremity edema, new skin rashes, nodules, fevers, or night sweats. • Five years earlier, the patient, who had been in the United States for 10 years, had been diagnosed with hypertension during a pregnancy. Her home medications included lisinopril, 40 mg daily, and hydrochlorothiazide, 25 mg daily, although she had not taken either for about 1 month prior to presentation. • She smokes about 3 cigarettes per day but denied use of alcohol, illicit drugs, or herbal supplements. Her mother died from complications of hypertension. There was no known family history of early CAD, sudden cardiac death, or rheumatologic diseases. • ASSESSMENT • The patient was afebrile with a regular heart rate of 85 beats per minute, equal in both arms blood pressure of 235/106 mmHg, a respiratory rate of 16 breaths per minute, and an oxygen saturation of 98% on ambient air. • She appeared comfortable and in no apparent distress. Her lungs were clear to auscultation bilaterally, and her jugular venous pressure was 10 cm of water. • A cardiac examination identified a regular rate and rhythm and a harsh III/VI systolic murmur at the apex with radiation to her axilla and back. • Her abdomen was mildly obese and nontender without hepatosplenomegaly. • No abdominal bruits were evident. Femoral pulses were absent bilaterally. The remainder of her examination was normal. • Laboratory analysis revealed a normal basic metabolic panel with no electrolyte abnormalities. The patient’s hemoglobin was 11.1 g/dL with a MCV of 90.8 mm3. Her Nterminal pro- BNP level was 2594 pg/mL (reference range, 0-449 pg/mL). • TSH was assessed at 1.65 mlU/L, and a urine pregnancy test was negative. Renin level was measured at 31 ng/mL/h (normal, <24 ng/ml/h). Urinalysis disclosed 2 protein and 3 blood. Urine toxicology testing was negative for cocaine, amphetamines, and other substances. • An ECG indicated marked left ventricular hypertrophy with repolarization abnormalities, while a chest x-ray showed a tortuous aorta and cardiomegaly. A work-up for secondary causes of hypertension ensued. • Transthoracic echocardiography demonstrated moderate concentric left ventricular hypertrophy and an estimated ejection fraction of 60%. Mild left atrial enlargement was detected. No significant valvular abnormalities were seen. The aortic root size was normal, measuring 2.6 cm at the sinuses. • Because femoral pulses were absent, additional imaging of the aorta was performed. • Figure 1 shows MRI of the abdomen and CTA of the abdomen and pelvis. • Figure 2 shows CTA of the chest . • A tuberculin PPD test showed 8-9 mm of induration in the setting of prior BCG vaccination. A serum interferon gamma release assay for Mycobacterium tuberculosis was positive. Rapid plasma regain testing for syphilis was negative. • An ACE level was within normal limits. Tests for ANA, p-ANCA and c-ANCA were negative. An ESR was 46 mm/hr , and CRP was elevated to 11.4 mg/L . The patient’s LDL level was 200 mg/dL , and the HDL level was 47 mg/dL . • Positron emission tomography (PET) was carried out with the tracer fludeoxyglucose18F (FDG) (Figure 3). • A core needle biopsy of a 1.4-cm, FDG-avid axillary lymph node showed no pathologic changes. An acid-fast bacilli (AFB) stain and culture were both negative. • DIAGNOSIS • Our patient was given a diagnosis of Takayasu arteritis with concomitant coronary artery disease. • This rare large-vessel vasculitis involves the aorta and its primary branches. It typically affects young women, with the greatest prevalence in Asia and South America. The pathogenesis of Takayasu arteritis remains poorly understood, but the disease is postulated to resemble giant cell arteritis with cell-mediated granulomatous changes. • Initially, inflammation occurs mostly in the adventitia and media of arteries, leading to thickening of the walls.3 With continued inflammation, reactive intimal hyperplasia and adventitial fibrosis may result in vascular stenoses. Reactive intimal hyperplasia also puts patients at risk for secondary atherosclerosis. • Authors found a high probability of Takayasu arteritis when 2 major criteria, 1 major and 2 minor criteria, or 4 minor criteria were fulfilled. • Our patient met 1 major criterion in that she had characteristic symptoms for at least 1 month. She also fulfilled 3 minor criteria: high ESR , hypertension, and a descending thoracic aorta lesion . • Giant cell arteritis is the other most common cause of large vessel vasculitis but this was an unlikely diagnosis, given our patient’s young age and the lack of ocular symptoms. • She had no upper respiratory tract symptoms to suggest granulomatosis with polyangiitis, and tests for antineutrophil cytoplasmic antibodies, proteinase 3 antibodies, and myeloperoxidase antibodies were negative. • Her negative rapid plasma reagin test ruled out syphilitic aortitis; similarly, a normal angiotensin-converting enzyme level made sarcoidosis unlikely. • Notably, our patient’s PPD test and interferon gamma release assay were positive, raising the possibility of tuberculous aortitis. However, tuberculous involvement of large vessels is exceedingly rare, with its mention in the literature limited to case reports and case series . • Also, tuberculous aortitis is characterized almost always by aneurysm formation. It is usually secondary to contiguous spread from adjacent infected lymph nodes, or less commonly, it is due to disseminated tuberculosis with vascular seeding. • Although tuberculous aortitis could not be definitively ruled out in our patient, the absence of signs and symptoms of active tuberculosis, her imaging findings, and her subsequent improvement with immunosuppression made Takayasu arteritis the leading diagnosis. • Several studies have identified a possible association between Takayasu arteritis and tuberculosis.7-10 An increased frequency of positive tuberculin skin tests has been observed in Takayasu arteritis patients, although the association is difficult to prove because many affected patients live in endemic countries. • MANAGEMENT • Systemic corticosteroids are the backbone of therapy for active Takayasu arteritis. Other immunosuppressive agents, including azathioprine, cyclophosphamide, mycophenolate mofetil, and antagonists of tumor necrosis factor alpha often are used in combination with corticosteroids or as an alternative to corticosteroids, but there are no randomized clinical trials evaluating these regimens. • High-dose corticosteroids were administered with a plan to initiate infliximab after she completed 9 months of isoniazid for latent tuberculosis. She also underwent stenting of the significant descending aortic stenosis, a procedure that afforded marked improvement in her blood pressure control. Thank you