Case presentation

Emergency department course

A 25-year-old male with the sole medical history of psoriasis presented to the Emergency Department with a chief complaint of painless hematuria for one week, a single episode of epistaxis, and mild headache since that morning. The patient further reported left upper extremity paresthesia for the past week. On further history, he reported he has not seen a doctor since his last pediatrician visit six years earlier. He had no prior history of smoking or recreational drug or alcohol use. His vital signs upon arrival were notable for a blood pressure of 253/167 and a heart rate of 114. His SpO2 was 99%, and body temperature was 37.2 °C. An EKG was performed, notable for ST elevations in aVR and V1-V3, with diffuse ST depressions (Figure 1).

Figure 1: Initial EKG on arrivalBlack arrows indicate ST elevations; Red arrows indicate ST depressions

On physical exam, the patient was in no acute distress. Subjective decreased sensation in the left upper extremity was noted. However, there was 5/5 strength, which was symmetric in all four extremities; ambulation was with a steady gait, there was no dysmetria, and no pronator drift. Laboratory evaluation was notable for a creatinine of 2.18 with a blood urea nitrogen (BUN) of 28, urinalysis with RBC >182, and high-sensitivity troponins of 34, 35 (1 hour), and 38 (2 hours). CBC and LFTs were within normal limits. A second EKG done an hour and a half later showed no major changes (Figure 2). The patient underwent non-contrast CT of the head revealing no ​acute intracranial hemorrhage, territorial loss of gray-white matter differentiation, or mass effect. Given normal CT of the head and the reassuring neurological exam, there was lower suspicion for large-vessel occlusion. Thrombotic thrombocytopenic purpura was also excluded, as the patient had a normal hemoglobin, normal platelet count, normal bilirubin, and no skin findings. Similarly, hemolytic uremic syndrome was also excluded given no lab findings indicating hemolysis. A CT angiogram of the chest, abdomen, and pelvis was performed, with no acute aortic process or acute finding. He was diagnosed with hypertensive emergency. He was treated with labetalol 10 mg IV and a nicardipine infusion with a target goal of reducing his blood pressure by 20%. To trend the blood pressure, he was placed on a continuous monitor, measuring his blood pressure every 15 minutes after being started on the nicardipine infusion. Table 1 exhibits the charted blood pressure measurements. While in the emergency department, his blood pressure remained in the 190s/130s, and he was admitted to the cardiac intensive care unit (CCU).

Figure 2: Second EKG 1.5 hours after the initialBlack arrows indicate ST elevations; Red arrows indicate ST depressions

Length of Stay (hours: minutes) | Blood Pressure (mmHg)
00:00 | 253/167
00:59 | 231/156
2:02 | 220/149
2:38 | 191/133
2:57 | 202/135
3:06 | 192/128
3:37 | 215/147
4:22 | 202/145
4:37 | 198/136

CCU course

The patient’s troponin peaked at 75 on hospital day 2. Workup for secondary causes of hypertension was pursued; cortisol levels were not suggestive of Cushing’s, but RNA polymerase III (an auto-antibody specific for scleroderma with renal involvement) was positive, and his elevated creatinine and urine protein were suggestive of renal parenchymal disease. Further workup for glomerulonephropathy, including erythrocyte sedimentation rate, complement levels, HIV, hepatitis panel, anti-neutrophil cytoplasmic antibodies, double-stranded DNA, and anti-glomerular basement membrane, was negative. The patient underwent transthoracic echocardiography, which revealed concentric left ventricular hypertrophy, normal function in both ventricles, and a normal ejection fraction of 60%. Bilateral renal ultrasound did not reveal renal artery stenosis. A CT of the abdomen and pelvis did not reveal adrenal masses. He was diagnosed with grade 3 hypertensive retinopathy on fundoscopic exam. The patient underwent a renal biopsy on hospital day 5, which was complicated by a hematoma. The result was for renal thrombotic microangiopathy (TMA), which can be autoimmune-mediated, coagulation-mediated, drug- or toxin-induced, or metabolism-associated. On day 3, he began oral treatment with amlodipine, hydrochlorothiazide, and losartan. He was transitioned off the nicardipine infusion on hospital day 8 and transferred to the general medical floor on day 8.

Floor course

THE oral antihypertensives were continued, and he was discharged on hospital day 9 on amlodipine 10 mg daily, hydrochlorothiazide 50 mg daily, losartan 100 mg daily, carvedilol 25 mg BID, and eplerenone 50 mg BID. He was instructed to follow up on an outpatient basis with nephrology, cardiology, and ophthalmology.

Post-hospitalization

After discharge, the patient was compliant with follow-up and asymptomatic. Blood pressure was controlled at 110-120 systolic. Consideration was given to initiating ravulizumab or eculizumab for treatment of severe TMA, and he was to undergo genetic testing.