A 25-year-old woman comes to the office with a 3-month history of fatigue, myalgias, arthralgias, and a 5-kg (11-lb) weight loss. She has also recently experienced leg pain with activity, which resolves with rest. The patient has no other medical conditions and takes no medications. Temperature is 37.6 C (99.7 F), and blood pressure is 160/90 mm Hg in the right arm and 120/80 mm Hg in the left. A bruit is heard over the left subclavian artery. Left radial pulse and dorsalis pedis pulses in both legs are weak. The remainder of the examination shows no abnormalities. Laboratory results reveal a hemoglobin level of 9.8 g/dL and an erythrocyte sedimentation rate of 110 mm/hr. Which of the following most likely underlies this patient's current condition?

Question

A 25-year-old woman comes to the office with a 3-month history of fatigue, myalgias, arthralgias, and a 5-kg (11-lb) weight loss.  She has also recently experienced leg pain with activity, which resolves with rest.  The patient has no other medical conditions and takes no medications.  Temperature is 37.6 C (99.7 F), and blood pressure is 160/90 mm Hg in the right arm and 120/80 mm Hg in the left.  A bruit is heard over the left subclavian artery.  Left radial pulse and dorsalis pedis pulses in both legs are weak.  The remainder of the examination shows no abnormalities.  Laboratory results reveal a hemoglobin level of 9.8 g/dL and an erythrocyte sedimentation rate of 110 mm/hr.  Which of the following most likely underlies this patient's current condition?

Quizplus · Accepted Answer

11ec160b_38eb_03b2_9249_a76d945279dd_MD0001_00 This patient with lower extremity claudication (ie, exertional pain due to limited blood flow reserve) and constitutional symptoms has typical features of Takayasu arteritis (TA). TA is a chronic, large-artery vasculitis that primarily involves the aorta and its branches. TA predominantly affects women of reproductive age and is more common in Asians. Arterial narrowing in TA can manifest as audible bruits, blood pressure discrepancies, pulse deficits, and distal ulcerations. Characteristic findings on histopathology include mononuclear infiltrates and granulomatous inflammation of the vascular media, leading to arterial wall thickening and occlusion. The pathologic abnormalities in TA closely resemble giant cell (temporal) arteritis; however, TA primarily affects younger individuals, whereas giant cell arteritis occurs almost exclusively in patients age ≥50. (Choice A) Exaggerated vascular smooth muscle contraction (eg, in response to cold or vibration) causes Raynaud phenomenon. Symptoms are typically acute and episodic and manifest as a triphasic color change (pallor, cyanosis, erythema) in the distal extremities. This patient's progressive symptoms and proximal arterial findings are more consistent with TA. (Choice C) Atherosclerosis is characterized by lipid-rich deposits in large- and medium-sized arteries. It is common in older patients with cardiovascular risk factors (eg, smoking, hypertension, hyperlipidemia) but is rare in individuals as young as this patient. In addition, in contrast to this patient who has both upper and lower extremity abnormalities, upper extremity atherosclerosis is usually a late finding. (Choice D) Necrotizing inflammation of medium-sized arteries is seen in polyarteritis nodosa, which is characterized by multisystem involvement (eg, glomerulonephritis, skin lesions, neuropathy, bowel ischemia, myositis). This patient's bruits, blood pressure discrepancies, and pulse deficits indicate a large artery disorder. (Choice E) Thromboangiitis obliterans (Buerger disease) is a segmental, thrombosing vasculitis of medium- and small-sized arteries, principally the tibial and radial arteries. Manifestations are most noticeable in the distal extremities (eg, ulcerations), and the condition is almost always seen in association with heavy cigarette smoking. Educational objective: Takayasu arteritis is a chronic, large-artery vasculitis that primarily involves the aorta and its branches. It presents with constitutional (eg, fever, weight loss) and arterio-occlusive (eg, claudication, blood pressure discrepancies, pulse deficits) findings in patients age <40. Histopathology shows granulomatous inflammation of the vascular media. __________ References: Diagnostic and classification criteria of Takayasu arteritis. (http://www.ncbi.nlm.nih.gov/pubmed/24461381) Updates in pathophysiology, diagnosis and management of Takayasu arteritis. (http://www.ncbi.nlm.nih.gov/pubmed/27238990)

A 25-Year-Old Woman Comes to the Office with a 3-Month