Young‐Adult Hypertension as a Presentation of Familial Renal Disease

Abstract

A previously healthy 20-year-old woman presented to the hospital with a 2-day history of headache, nausea, vomiting, and a blood pressure (BP) of 240/150 mm Hg. She also described mild visual blurriness and transient numbness of the right upper extremity. Eight months before admission and prior to initiation of oral contraceptive pills (OCPs) (1 mg ethynodiol, 35 µg estradiol) for control of mild metromenorrhagia, recorded BPs were ≈120/70 mm Hg. After 2 days of treatment, BP improved to 150/100 mm Hg and the patient was discharged on metoprolol 50 mg b.i.d. A negative workup for secondary etiologies included 24-hour urine collections for vanillylmandelic acid assay, metanephrines, and catecholamines, a postcaptopril renal scan, and an abdominal magnetic resonance imaging scan. A brain magnetic resonance imaging scan was normal. Laboratory testing revealed blood urea nitrogen 25, creatinine 1.6, and urinalysis with 1+ protein. OCPs were discontinued, and she was referred to the hypertension clinic.Physical examination revealed an obese white female with body mass index 38, BP 164/96 mm Hg. There was no edema or striae, peripheral pulses were intact without bruits, and there was no retinopathy. Family history was significant; the patient's maternal grandmother had hypertension without known renal disease. Her mother was several years post renal transplant for end-stage renal disease (ESRD) secondary to biopsy-proven focal segmental glomerulosclerosis (FSGS). Her mother had renal disease with proteinuria and azotemia without hypertension and was not obese. Her father and 15-year-old sister were normal. Illicit drug use was denied.Additional workup proceeded with a normal renal artery magnetic resonance angiography study, antinuclear antibody negative, human immunodeficiency virus negative, sedimentation rate of 36, negative hepatitis serology, a normal 24-hour urine free cortisol collection, and normal renal ultrasonography. Six months following OCP cessation, ongoing treatment with hydrochlorothiazide, metoprolol, and lisinopril resulted in blood pressure of 140/100 mm Hg. Serum creatinine levels were consistently 1.8-2.0 mg/dL with a creatinine clearance of 73 cc/min, and a 24-hour urine protein collection was 2.5 g. The patient was referred for renal biopsy. Light microscopy and electron microscopy findings were consistent with focal segmental glomerulosclerosis (Figures 1 and 2). DISCUSSIONInitial suspicion was that this patient's hypertension was related to OCPs because of the documented onset on antimetromenorrhagia therapy. Most often OCP-related BP elevations are between 4 and 9 mm Hg systolic although diastolic elevations can be seen. 1 Severe elevation in previously normotensive persons is a rare observation. 2 BP elevations on OCPs most often return to pretreatment levels following 3-6 months of cessation 1 but remained elevated for this patient. Malignant hypertension on OCPs can occur 2 in association with nephrotic-range proteinuria and azotemia. However, the family history of FSGS in the context of ...