Clinical Review & Education
JAMA Surgery Clinical Challenge
Progressive Lower Extremity Pain Dustin Y. Yoon, MD; Andrew W. Hoel, MD
Figure 1. Magnetic resonance angiography of the abdomen and pelvis.
A 39-year-old active woman presented with a progressive 6-month history of bilateral lower extremity pain after walking 1 block or ascending a flight of stairs. The pain was aching, relieved with rest, and primarily in her buttocks and thighs bilaterally. The patient denied history of prior abdominal or lower extremity surgery, trauma, or cardiovascular problems. Review of systems and family history revealed nothing remarkable. Physical examination Quiz at jamasurgery.com revealed normal sinus rhythm and normal blood pressure. She had no abdominal tenderness and no palpable masses. Femoral pulses were weakly palpable and symmetric, and pedal pulses were not palpable. Laboratory findings were unremarkable except for an elevated erythrocyte sedimentation rate (ESR) (34 mm/h). Blood and urine cultures were negative. Noninvasive arterial flow studies demonstrated an ankle brachial index of 0.60 on the right and 0.62 on the left with biphasic waveforms in the femoral, popliteal, and tibial vessels. Subsequent magnetic resonance angiography (MRA) of the chest, abdomen, and pelvis showed mild stenosis of bilateral subclavian arteries and an abnormal-appearing infrarenal aorta (Figure 1).
jamasurgery.com
WHAT IS YOUR DIAGNOSIS?
A. Middle aortic syndrome B. Inflammatory aneurysm C. Giant cell arteritis D. Takayasu arteritis
(Reprinted) JAMA Surgery January 2016 Volume 151, Number 1
Copyright 2016 American Medical Association. All rights reserved.
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81
Clinical Review & Education JAMA Surgery Clinical Challenge
Answer D. Takayasu arteritis
Discussion The patient was a young woman with claudication and diminished lower extremity pulses. She has a mildly elevated ESR and hemodynamically significant suprainguinal occlusive disease. Imaging revealed subclavian artery and infrarenal aortic stenoses. These findings are suggestive of Takayasu arteritis (TA).1 Takayasu arteritis is an immune-mediated inflammatory arteritis of the aorta and its major branches occurring before the fourth decade of life with a female to male ratio of approximately 3:1.2 Concomitant cardiac and visceral artery involvement is also observed in some patients. The incidence of TA varies widely by geographic location. In Asia, there are approximately 150 new cases per year.3 The pathogenesis for TA remains elusive, with studies showing not only genetic predispositions but also immune-mediated mechanisms of inflammation and endothelial damage.4 Immunohistochemical studies reveal dense fibrotic tissue of all 3 layers of the vessel wall. Aneurysmal disease in TA is not uncommon, with apparent destruction of the elastic media of the involved artery.5 The consensus criteria for diagnosis is still debated, but currently there are 2 major criteria used: (1) American College of Rheumatology Criteria6; and (2) Modified Ishikawa Criteria.7 Both emphasize abnormalities of the aorta and major branches, extremity claudication, and imaging abnormalities. Computed tomography (CT) angiography, MRA, and digital subtraction angiography have all been used to diagnose TA. Positron emission tomography CT has also been used to visualize inflammation within the arterial wall. There are no specific laboratory markers for TA, but in the active phase, ESR, C-reactive protein, interleukin, and matrix metalloproteinase values may be elevated.3,8 Glucocorticoids are the first-line treatments for TA. Twentypercent of patients have self-limited, monophasic illness that does not need further immunomodulating agents.9 Relapse, however, can occur even during immunosuppressive therapy in 50% of patients.2 Surgical treatment is reserved for those for whom conservative treatARTICLE INFORMATION Author Affiliations: Division of Vascular Surgery, Northwestern University Feinberg School of Medicine, Chicago, Illinois. Corresponding Author: Andrew W. Hoel, MD, Division of Vascular Surgery, Northwestern Memorial Hospital, Arkes Family Pavilion, Ste 650, 676 N Saint Clair St, Chicago IL 60611 ([email protected]). Section Editor: Pamela A. Lipsett, MD, MHPE. Published Online: November 11, 2015. doi:10.1001/jamasurg.2015.1478. Conflict of Interest Disclosures: None reported. REFERENCES 1. Sharma BK, Siveski-Iliskovic N, Singal PK. Takayasu arteritis may be underdiagnosed in North America. Can J Cardiol. 1995;11(4):311-316.
82
Figure 2. Postoperative magnetic resonance angiography of the aorta demonstrating suprarenal to left common iliac artery bypass.
ment fails, including patients with lifestyle-limiting claudication, cardiac ischemia, hypertension due to renal artery stenosis, and aneurysmal degeneration. In general, endovascular interventions have not demonstrated long-term efficacy, and open surgical treatment remains the gold standard.10 The present patient had progression of symptoms, in spite of treatment with steroids and azathioprine, leading to significant functional limitation. One year after diagnosis, after a decrease in ESR and stable findings on imaging studies demonstrated that quiescence had been achieved, she underwent a suprarenal aorta to common iliac artery bypass (Figure 2). She had an uneventful postoperative course with complete resolution of symptoms.
2. Kerr GS, Hallahan CW, Giordano J, et al. Takayasu arteritis. Ann Intern Med. 1994;120(11):919-929. 3. Kerr GS. Takayasu’s arteritis. Rheum Dis Clin North Am. 1995;21(4):1041-1058. 4. Glebova NO, Abularrage CJ. Takayasu’s disease. In: Cronenwett JL, Johnston KW, eds. Rutherford’s Vascular Surgery. 8th ed. Philadelphia, PA: Elsevier Saunders; 2014:1187-1202. 5. Numano F, Kishi Y, Tanaka A, Ohkawara M, Kakuta T, Kobayashi Y. Inflammation and atherosclerosis. Atherosclerotic lesions in Takayasu arteritis. Ann N Y Acad Sci. 2000;902:65-76. 6. Arend WP, Michel BA, Bloch DA, et al. The American College of Rheumatology 1990 criteria for the classification of Takayasu arteritis. Arthritis Rheum. 1990;33(8):1129-1134.
7. Sharma BK, Jain S, Suri S, Numano F. Diagnostic criteria for Takayasu arteritis. Int J Cardiol. 1996; 54(suppl):S141-S147. 8. Matsuyama A, Sakai N, Ishigami M, et al. Matrix metalloproteinases as novel disease markers in Takayasu arteritis. Circulation. 2003;108(12): 1469-1473. 9. Hoffman GS. Takayasu arteritis: lessons from the American National Institutes of Health experience. Int J Cardiol. 1996;54(suppl):S99-S102. 10. Fava MP, Foradori GB, García CB, et al. Percutaneous transluminal angioplasty in patients with Takayasu arteritis: five-year experience. J Vasc Interv Radiol. 1993;4(5):649-652.
JAMA Surgery January 2016 Volume 151, Number 1 (Reprinted)
Copyright 2016 American Medical Association. All rights reserved.
Downloaded From: http://archsurg.jamanetwork.com/ by a DALHOUSIE UNIVERSITY-DAL-11762 User on 01/31/2016
jamasurgery.com
JAMA Surgery Clinical Challenge
Progressive Lower Extremity Pain Dustin Y. Yoon, MD; Andrew W. Hoel, MD
Figure 1. Magnetic resonance angiography of the abdomen and pelvis.
A 39-year-old active woman presented with a progressive 6-month history of bilateral lower extremity pain after walking 1 block or ascending a flight of stairs. The pain was aching, relieved with rest, and primarily in her buttocks and thighs bilaterally. The patient denied history of prior abdominal or lower extremity surgery, trauma, or cardiovascular problems. Review of systems and family history revealed nothing remarkable. Physical examination Quiz at jamasurgery.com revealed normal sinus rhythm and normal blood pressure. She had no abdominal tenderness and no palpable masses. Femoral pulses were weakly palpable and symmetric, and pedal pulses were not palpable. Laboratory findings were unremarkable except for an elevated erythrocyte sedimentation rate (ESR) (34 mm/h). Blood and urine cultures were negative. Noninvasive arterial flow studies demonstrated an ankle brachial index of 0.60 on the right and 0.62 on the left with biphasic waveforms in the femoral, popliteal, and tibial vessels. Subsequent magnetic resonance angiography (MRA) of the chest, abdomen, and pelvis showed mild stenosis of bilateral subclavian arteries and an abnormal-appearing infrarenal aorta (Figure 1).
jamasurgery.com
WHAT IS YOUR DIAGNOSIS?
A. Middle aortic syndrome B. Inflammatory aneurysm C. Giant cell arteritis D. Takayasu arteritis
(Reprinted) JAMA Surgery January 2016 Volume 151, Number 1
Copyright 2016 American Medical Association. All rights reserved.
Downloaded From: http://archsurg.jamanetwork.com/ by a DALHOUSIE UNIVERSITY-DAL-11762 User on 01/31/2016
81
Clinical Review & Education JAMA Surgery Clinical Challenge
Answer D. Takayasu arteritis
Discussion The patient was a young woman with claudication and diminished lower extremity pulses. She has a mildly elevated ESR and hemodynamically significant suprainguinal occlusive disease. Imaging revealed subclavian artery and infrarenal aortic stenoses. These findings are suggestive of Takayasu arteritis (TA).1 Takayasu arteritis is an immune-mediated inflammatory arteritis of the aorta and its major branches occurring before the fourth decade of life with a female to male ratio of approximately 3:1.2 Concomitant cardiac and visceral artery involvement is also observed in some patients. The incidence of TA varies widely by geographic location. In Asia, there are approximately 150 new cases per year.3 The pathogenesis for TA remains elusive, with studies showing not only genetic predispositions but also immune-mediated mechanisms of inflammation and endothelial damage.4 Immunohistochemical studies reveal dense fibrotic tissue of all 3 layers of the vessel wall. Aneurysmal disease in TA is not uncommon, with apparent destruction of the elastic media of the involved artery.5 The consensus criteria for diagnosis is still debated, but currently there are 2 major criteria used: (1) American College of Rheumatology Criteria6; and (2) Modified Ishikawa Criteria.7 Both emphasize abnormalities of the aorta and major branches, extremity claudication, and imaging abnormalities. Computed tomography (CT) angiography, MRA, and digital subtraction angiography have all been used to diagnose TA. Positron emission tomography CT has also been used to visualize inflammation within the arterial wall. There are no specific laboratory markers for TA, but in the active phase, ESR, C-reactive protein, interleukin, and matrix metalloproteinase values may be elevated.3,8 Glucocorticoids are the first-line treatments for TA. Twentypercent of patients have self-limited, monophasic illness that does not need further immunomodulating agents.9 Relapse, however, can occur even during immunosuppressive therapy in 50% of patients.2 Surgical treatment is reserved for those for whom conservative treatARTICLE INFORMATION Author Affiliations: Division of Vascular Surgery, Northwestern University Feinberg School of Medicine, Chicago, Illinois. Corresponding Author: Andrew W. Hoel, MD, Division of Vascular Surgery, Northwestern Memorial Hospital, Arkes Family Pavilion, Ste 650, 676 N Saint Clair St, Chicago IL 60611 ([email protected]). Section Editor: Pamela A. Lipsett, MD, MHPE. Published Online: November 11, 2015. doi:10.1001/jamasurg.2015.1478. Conflict of Interest Disclosures: None reported. REFERENCES 1. Sharma BK, Siveski-Iliskovic N, Singal PK. Takayasu arteritis may be underdiagnosed in North America. Can J Cardiol. 1995;11(4):311-316.
82
Figure 2. Postoperative magnetic resonance angiography of the aorta demonstrating suprarenal to left common iliac artery bypass.
ment fails, including patients with lifestyle-limiting claudication, cardiac ischemia, hypertension due to renal artery stenosis, and aneurysmal degeneration. In general, endovascular interventions have not demonstrated long-term efficacy, and open surgical treatment remains the gold standard.10 The present patient had progression of symptoms, in spite of treatment with steroids and azathioprine, leading to significant functional limitation. One year after diagnosis, after a decrease in ESR and stable findings on imaging studies demonstrated that quiescence had been achieved, she underwent a suprarenal aorta to common iliac artery bypass (Figure 2). She had an uneventful postoperative course with complete resolution of symptoms.
2. Kerr GS, Hallahan CW, Giordano J, et al. Takayasu arteritis. Ann Intern Med. 1994;120(11):919-929. 3. Kerr GS. Takayasu’s arteritis. Rheum Dis Clin North Am. 1995;21(4):1041-1058. 4. Glebova NO, Abularrage CJ. Takayasu’s disease. In: Cronenwett JL, Johnston KW, eds. Rutherford’s Vascular Surgery. 8th ed. Philadelphia, PA: Elsevier Saunders; 2014:1187-1202. 5. Numano F, Kishi Y, Tanaka A, Ohkawara M, Kakuta T, Kobayashi Y. Inflammation and atherosclerosis. Atherosclerotic lesions in Takayasu arteritis. Ann N Y Acad Sci. 2000;902:65-76. 6. Arend WP, Michel BA, Bloch DA, et al. The American College of Rheumatology 1990 criteria for the classification of Takayasu arteritis. Arthritis Rheum. 1990;33(8):1129-1134.
7. Sharma BK, Jain S, Suri S, Numano F. Diagnostic criteria for Takayasu arteritis. Int J Cardiol. 1996; 54(suppl):S141-S147. 8. Matsuyama A, Sakai N, Ishigami M, et al. Matrix metalloproteinases as novel disease markers in Takayasu arteritis. Circulation. 2003;108(12): 1469-1473. 9. Hoffman GS. Takayasu arteritis: lessons from the American National Institutes of Health experience. Int J Cardiol. 1996;54(suppl):S99-S102. 10. Fava MP, Foradori GB, García CB, et al. Percutaneous transluminal angioplasty in patients with Takayasu arteritis: five-year experience. J Vasc Interv Radiol. 1993;4(5):649-652.
JAMA Surgery January 2016 Volume 151, Number 1 (Reprinted)
Copyright 2016 American Medical Association. All rights reserved.
Downloaded From: http://archsurg.jamanetwork.com/ by a DALHOUSIE UNIVERSITY-DAL-11762 User on 01/31/2016
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