P0629 - A Teenager with Hypertension: A common problem leading to an Uncommon Diagnosis

Introduction:
Takayasu Arteritis (TA) is a rare diagnosis mainly affecting women aged 15-30 years of Asian or Indian descent, with an incidence of 1.2-2.6 per million annually. This case describes the diagnosis of TA in a young Caucasian female with no known Asian/Indian ancestry and no significant family history who presented with new-onset hypertension.

Case Description:
A 16-year-old previously healthy female presented to the pediatric emergency department due to “abnormal lab values”. The patient was initially seen 2 weeks prior after becoming lightheaded while participating in an active shooter drill at school, at which time her blood pressure (BP) was found to be elevated to 180/100 mmHg. Associated symptoms included palpitations, dyspnea on exertion, weight loss, bilateral shoulder pain, night sweats, fatigue, and frontal headaches with photophobia. Labs done by her PCP were significant for mild leukocytosis, microcytic anemia, and thrombocytosis along with mildly elevated creatinine, hypoalbuminemia, and proteinuria resulting in referral to the emergency department. Initial labs, labs on presentation, and follow up labs are included in Table 1. On presentation, physical exam was significant for BP of 168/68 mmHg, heart rate 126 bpm, and temperature 38.2 degrees Celsius. She was a pale-appearing, thin female with a 2/6 systolic murmur with radiation to carotids and subclavian, decreased right brachial pulse, 3/6 abdominal bruit, sharp optic discs, strength 5/5 throughout and no edema, rash or arthritis. Renal ultrasound revealed an enlarged right kidney with low-normal size left kidney. Under guidance of nephrology and rheumatology, she was admitted and underwent CT Angiogram revealing large and medium vessel vasculitis with diffuse wall thickening and multifocal luminal stenosis. The areas of most severe arterial narrowing (luminal diameter of 1-2 mm) were the right subclavian artery, bilateral carotid arteries, superior mesenteric artery, inferior right renal artery, and left renal artery, consistent with a diagnosis of TA (Figure 1). Initial treatment in hospital included pulse dose IV methylprednisolone and anti-hypertensive medication with prolonged oral steroid taper at discharge. Methotrexate and infliximab were added as steroid-sparing immunosuppressant agents. BP was difficult to control and she is now treated with metoprolol, hydrochlorothiazide, clonidine and, most recently added, enalapril, with significant improvement in BP. There are plans for definitive intervention for renal vascular hypertension when inflammation is better controlled. Etiology of nephrotic range proteinuria is unclear. Renal biopsy has been delayed secondary to hypertension and high risk of procedure.

Discussion/Importance:
Though rare, TA is a diagnostic consideration for new-onset hypertension in a teenager or young adult. Systemic inflammation and hypertension caused by TA is managed with anti-hypertensives, glucocorticoids, and immunosuppressant therapies including methotrexate and a TNF blocking agent such as infliximab. Proteinuria is unusual for TA thus etiology is still unclear.