Hypertensive crisis in a 39-year-old Haitian woman with a history of hypertension proved secondary to an underlying chronic disease. Having arrived in the emergency department for evaluation of a severe headache and shortness of breath, she added that, for several months, she had been experiencing chest pain, both at rest and with exertion. Episodes lasted for 10-15 minutes and were substernal and squeezing in nature, often reaching 10/10 in severity. These were intermittently associated with shortness of breath 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. A smoker with a 10 pack-year history, she calculated her current use at about 3 cigarettes per day. She denied use of alcohol, illicit drugs, or herbal supplements. Her mother died from complications of hypertension. There was no known family history of early coronary artery disease, sudden cardiac death, or rheumatologic diseases.
Assessment
On physical examination, the patient was afebrile with a regular heart rate of 85 beats per minute, blood pressure of 235/106 mmHg, a respiratory rate of 16 breaths per minute, and an oxygen saturation of 98% on ambient air. Blood pressure was equal in both arms. 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 mean corpuscular volume of 90.8 μm3. Her N-terminal pro-brain natriuretic peptide level was 2594 pg/mL (reference range, 0-449 pg/mL). Thyroid stimulating hormone 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 electrocardiogram 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. Magnetic resonance imaging of the abdomen revealed diffuse ectasia of the entire descending aorta. Severe focal stenosis was apparent at the level of the aortic hiatus; an internal diameter of 1 × 1 cm was concerning for aortitis. Computed tomography angiography (CTA) of the abdomen and pelvis confirmed a circumferentially thickened aortic wall with diffuse ectasia, as well as significant stenosis with collaterals to the epigastric arteries. CTA of the chest suggested vasculitis of the coronary arteries, given the diffuse bead-like pattern of multiple critical focal stenoses of greater than 70% throughout the entire left anterior descending artery, left circumflex artery, and right coronary artery.
Further testing was ordered to look for infectious etiologies of aortitis. A tuberculin purified protein derivative (PPD) test showed 8-9 mm of induration in the setting of prior Bacillus Calmette-Guérin vaccination. A serum interferon gamma release assay for Mycobacterium tuberculosis was positive. Rapid plasma regain testing for syphilis was negative.
An angiotensin-converting enzyme level was within normal limits. Tests for antinuclear antibodies, antineutrophil cytoplasmic antibodies, and myeloperoxidase and proteinase 3 antibodies were negative. An erythrocyte sedimentation rate was accelerated at 46 mm/hr (reference range, 0-18 mm/hr), and C-reactive protein was elevated to 11.4 mg/L (reference range, 0-3 mg/L). The patient’s low-density lipoprotein level was 200 mg/dL (reference range, 50-129 mg/dL), and the high-density lipoprotein level was 47 mg/dL (reference range, 40-80 mg/dL).
Positron emission tomography (PET) was carried out with the tracer fludeoxyglucose-18F (FDG). Areas of increased tracer uptake suggest active inflammation. Because none were found in the aorta, active aortitis was ruled out. However, nonspecific increased tracer uptake within the bone marrow of the patient’s spine and pelvis, the bilateral inguinal and axillary lymph nodes, and the spleen, was a potential indication of diffuse inflammation. 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.
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-Fátima Rodriguez, MD, MPH, Kathleen O. Degnan, MD, Prashant Nagpal, MD, Ron Blankstein, MD, Marie D. Gerhard-Herman, MD
This article originally appeared in the December 2015 issue of The American Journal of Medicine.
