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

All her routine laboratory values were completely normal six months ago. Her blood pressure during her previous visits was always

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
 
All her routine laboratory values were completely normal six months ago.  Her blood pressure during her previous visits was always <140/80 mm Hg.  What is the most likely diagnosis of this patient?

Quizplus · Accepted Answer

Explanation: This patient's elevated blood pressure is clearly an acute change based on her prior blood pressure measurements. The most likely diagnosis in this case is scleroderma renal crisis. Underlying scleroderma should be considered in this patient given her history of Raynaud phenomenon and GERD. Scleroderma (systemic sclerosis) is characterized by the abnormal deposition of collagen in multiple organ systems. The majority of the patients have underlying renal involvement, but overt symptoms of scleroderma renal disease are seen less commonly. Thickening of the vessel wall and narrowing of the vascular lumen in the renal arterioles result in ischemia, which activates the renin-angiotensin system. This results in hypertension, which is often as severe as malignant hypertension, as seen in this patient with CNS symptoms and papilledema. It is, therefore, important to monitor the blood pressure in scleroderma patients, and the presence of hypertension should alert the physician to the possibility of scleroderma renal crisis. Acute renal failure is also typical of scleroderma renal crisis and is likely secondary to a combination of severe hypertension superimposed on the abnormal renal vasculature. (Choice A) Another possible cause for a sudden onset of renal crisis is renal artery stenosis secondary to atherosclerosis. However, atherosclerosis is unlikely in a young patient, such as this one, without atherosclerotic risk factors. A bruit is typically present in patients with atherosclerotic disease involving the renal arteries, but a bruit is not seen with scleroderma involvement of the kidneys because only the small arterioles are affected. (Choice B) Fibromuscular dysplasia can be a cause of renal artery stenosis in young patients. However, the presence of Raynaud phenomenon and GERD make scleroderma the more likely diagnosis. (Choice C) Sudden-onset renal failure can be seen in hemolytic uremic syndrome (HUS); however, it is predominantly manifested by hematological abnormalities, (i.e., thrombocytopenia and microangiopathic hemolytic anemia), and there is usually an accompanying history of prior diarrhea. HUS is commonly seen in children. (Choice D) Pheochromocytoma is usually associated with episodes of tachycardia, palpitations, headache, and hypertension. Educational objective: Scleroderma renal crisis presents with severe hypertension and renal failure in a patient with evidence of underlying scleroderma, such as Raynaud phenomenon and GERD. References: Scleroderma renal crisis: a rare but severe complication of systemic sclerosis. (http://www.ncbi.nlm.nih.gov/pubmed/20012923)

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