P0408 - Secondary Hypertension in Preventive Cardiology: A Rare Rheumatologic Disease

A previously healthy 16 year old black female presented with hypertension, 20 pound weight loss, fatigue, exercise intolerance and epistaxis. She described a throbbing headache for ten days. Systolic BP (SBP) was 110mmHg five months prior. Exam revealed cachexia, tachycardia, normal rhythm, 2/6 blowing systolic murmur LLSB, and an S3 gallop. Pulses: right brachial faint, left brachial 1+ and bilateral femoral 3+. SBP was 130mmHg in the left upper extremity, unable to be auscultated on the right, and 260mmHg in right lower extremity. EKG: sinus tachycardia, 109bpm, nonspecific ST-T wave changes in lateral precordial leads, borderline left ventricular hypertrophy (LVH). Echo: mild concentric LVH, diminished systolic function, LVEF 35-40%. Renal US: increased echogenicity of renal parenchyma bilaterally, and small left kidney. Labs: anemia, hypokalemia, thrombocytosis, elevated inflammatory markers, severe proteinuria and elevated direct serum renin (Table 1).
Upon PICU admission for hypertensive emergency, a nicardipine drip was started. Abdominal/chest/neck MRA showed edema and increased T2 signal diffusely involving the aortic arch vessels, thoracic and abdominal aorta. Wall thickening of the aortic arch extended to both subclavian arteries, and right common carotid artery. Wall thickening and inflammation of the proximal bilateral renal arteries with stenosis of the left renal artery (1.5 mm diameter) and proximal involvement of common iliac and external iliac arteries were noted. Brain MRA showed flattening/minimal protrusion of the optic discs.

She was diagnosed with active Takayasu Arteritis Type 5 based on the 1990 American College of Rheumatology criterion, secondary hyperaldosteronism, hypertensive cardiomyopathy and idiopathic intracranial hypertension. Initial treatment was solumedrol 30 mg/kg, nicardipine drip, intravenous iron and electrolyte replacement. She was transitioned to prednisone, infliximab infusions every two weeks, lisinopril, amlodipine, ferrous sulfate,and potassium chloride and discharged on day six. Repeat MRA six weeks later showed improvement of large vessel arterial wall thickening, persistent moderate-to-severe stenosis of left subclavian artery, mild stenosis of superior mesenteric artery, and severe stenosis of left renal artery with progressing renal atrophy. Repeat echo showed LVEF 57% and mild LVH. Renal scan showed decreased perfusion and function of the left kidney (11%). Oral Methotrexate was added to prevent development of human anti-chimeric antibodies. The patient reported minimal fatigue, increased appetite, headache resolution and improved exercise tolerance.
Young patients with unexplained hypertension benefit from a hypertension specialist with access to other sub-specialties and diagnostic modalities. Takayasu’s arteritis is rare, often with a long period from initial presentation until diagnosis. Patients with profound hypertension may be missed if diastolic pressures are mistaken as systolic pressures. History and exam techniques including systemic symptom inquiries, pulses in all extremities, further investigation when diastolic pressures are not found and BP in multiple extremities may help identify these rare cases.