A 36-year-old African American woman comes to the emergency department and complains of a severe, throbbing headache that began several hours ago. She also has acute onset of blurred vision and nausea. She took acetaminophen, which did not provide any relief. She was diagnosed with Raynaud phenomena a few years ago. She recently started having heartburn, for which she takes over-the-counter ranitidine. Her temperature is 36.7C (98.0F), blood pressure is 200/110 mm Hg, and pulse is 94/min. Pulse oximetry showed 94% in room air. There is no abdominal bruit. Ophthalmologic examination demonstrates bilateral papilledema. The laboratory results are as follows: CBC
Serum
Which of the following is the most appropriate pharmacotherapy?

Question

A 36-year-old African American woman comes to the emergency department and complains of a severe, throbbing headache that began several hours ago.  She also has acute onset of blurred vision and nausea.  She took acetaminophen, which did not provide any relief.  She was diagnosed with Raynaud phenomena a few years ago.  She recently started having heartburn, for which she takes over-the-counter ranitidine.  Her temperature is 36.7C (98.0F), blood pressure is 200/110 mm Hg, and pulse is 94/min.  Pulse oximetry showed 94% in room air.  There is no abdominal bruit.  Ophthalmologic examination demonstrates bilateral papilledema.  The laboratory results are as follows: CBC
 Serum
 Which of the following is the most appropriate pharmacotherapy?

Quizplus · Accepted Answer

Explanation: ACE inhibitors are the agents of choice in the treatment of scleroderma renal crisis since these reverse the angiotensin-induced vasoconstriction. Most patients respond favorably if these drugs are used promptly after the diagnosis, and the goal should be to reduce blood pressure to baseline over 72 hours. The ACE inhibitor of choice in these patients is captopril, given that it has a relatively short time to onset and is the agent with which there is the most clinical experience for scleroderma renal crisis. ACE inhibitors also alleviate renal failure in the majority of patients in the long term, although they may temporarily worsen renal failure in the short term. Since ACE inhibitors result in efferent arteriole vasodilation, there may be a mild drop in GFR and subsequent worsening of creatinine, which is already elevated in this patient as is commonly seen in scleroderma renal crisis. Creatinine should be closely monitored in these patients, but generally any increase in creatinine is mild and does not warrant cessation of the ACE inhibitor. Although ACE inhibitors are generally avoided in most patients with acute renal failure, scleroderma renal crisis is an exceptionto this general rule as long as renal function is closely monitored. In addition, in patients such as this one with CNS manifestations (eg, malignant hypertension), intravenous nitroprusside is added to acutely lower the blood pressure. This should be done cautiously as a sudden reduction of blood pressure is not favorable because it can further compromise renal perfusion and result in acute tubular necrosis; in addition, nitroprusside use should be very carefully monitored in the setting of renal injury (due to the risk of cyanide poisoning). Nitroprusside should be used for a short duration but is generally necessary in patients with scleroderma renal crisis who have CNS manifestations in order to gain control of the blood pressure acutely as the captopril takes effect. (Choice A) Calcium channel blockers may be used as a second-line agent in addition to captopril in the management of chronic hypertension in patients with scleroderma. Calcium channel blockers may also be of benefit for treatment of Raynaud phenomenon in these patients. (Choice B) Lasix would not be helpful for this patient as volume overload is not the underlying problem. (Choice D) Intravenous nitroprusside is frequently utilized in the management of malignant hypertension. However, starting captopril should be a priority in these patients as this is the agent of choice for treating scleroderma renal crisis. (Choice E) Clonidine patch will take longer to start its effect. Educational objective: ACE inhibitors, particularly captopril, are the drugs of choice for the treatment of hypertension in scleroderma renal crisis. The addition of intravenous medications such as nitroprusside is needed if CNS manifestations or papilledema is present. References: Long-term outcomes of scleroderma renal crisis. (http://www.ncbi.nlm.nih.gov/pubmed/11033587)

A 36-Year-Old African American Woman Comes to the Emergency Department