eMedicine Specialties > Cardiology > Peripheral Vascular Disease

Aortitis: Differential Diagnoses & Workup

Author: Masato Okada, MD, FACP, FACR, FAAAAI, Consulting Staff, Section of Allergy and Rheumatology, Department of Medicine, St Luke's International Hospital, Tokyo
Coauthor(s): Justin D Pearlman, MD, PhD, ME, MA, Director of Dartmouth Advanced Imaging Center, Professor of Medicine, Professor of Radiology, Adjunct Professor, Thayer Bioengineering and Computer Science, Dartmouth-Hitchcock Medical Center
Contributor Information and Disclosures

Updated: Mar 25, 2008

Differential Diagnoses

Behcet Disease
Renal Artery Aneurysm
Churg-Strauss Syndrome
Renal Artery Stenosis
Giant Cell Arteritis
Sarcoidosis
Inflammatory Bowel Disease
Syphilis
Kawasaki Disease
Systemic Lupus Erythematosus
Mycosis Fungoides
Takayasu Arteritis
Paraneoplastic Syndromes
Tuberculosis
Polyarteritis Nodosa
Wegener Granulomatosis
Renal Arteriovenous Malformation

Other Problems to Be Considered

Scalenus anticus syndrome and chronic regional pain syndrome/sympathetic dysfunction can cause asymmetry of pulses. With scalenus anticus, the neck muscles have focal tenderness, and position of neck and arm can change the pulse strength. With sympathetic dysfunction, a thermal map may identify >1°C difference between the arms exacerbated by exposure to cold or wind, and bone scan may show focal activity in small bones of the wrist. In advanced cases, asymmetry of nail and hair growth may be noted.

To make the diagnosis of arteritis at the early stage, a high index of suspicion for vasculitis is necessary, especially in patients with nonspecific inflammatory manifestations such as fatigue, malaise, joint aches, and low-grade fever but no specific clinical picture of other autoimmune diseases or infections. High index of suspicion is essential, not just in young women—recently, a highly esteemed senior American rabbi died from severe vasculitis because the correct diagnosis was not considered in time. Carotidynia presenting as neck pain can be an important clue. C-reactive protein (CRP) level and erythrocyte sedimentation rate (ESR) often are elevated, which supports the presence of an ongoing inflammatory process.

In the prepulseless phase, vascular changes may be too subtle to cause obvious extremity ischemia or arm claudication. With awareness of the disease, however, careful examination of the arteries at this stage may lead to the detection of reduction in one or more pulses; differences in blood pressure between the arms; or bruits over the neck, supraclavicular areas, axillae, or abdomen.

American College of Rheumatology published the classification criteria of Takayasu arteritis as follows: (1) age 40 years or younger at disease onset; (2) claudication of extremities; (3) decreased brachial artery pulse; (4) systolic blood pressure difference of greater than 10 mm Hg between arms; (5) bruit over subclavian arteries or abdominal aorta; and (6) arteriographic narrowing or occlusion of the entire aorta, its primary branches, or large arteries in the proximal upper or lower extremities that is not caused by arteriosclerosis, fibromuscular dysplasia, or similar causes.

The presence of at least 3 criteria reportedly yields a sensitivity of 90.5% and a specificity of 97.8%, although, being a clinical syndrome, the estimate of sensitivity is questionable.

Fibromuscular dysplasia, Ehlers-Danlos syndrome, and Marfan syndrome are associated with noninflammatory lesions that can mimic arteritis in angiographic findings. Fibromuscular dysplasia most commonly affects the renal arteries and leads to stenotic changes. Other sites, including carotid arteries and mesenteric arteries, can be involved, but the lesion usually is more focal. Ehlers-Danlos syndrome and Marfan syndrome also cause aortic aneurysm and aortic root dilatation, respectively. Their systemic manifestations and lack of typical clinical symptoms of Takayasu arteritis may help differentiation.

Infectious diseases, including syphilis, tuberculosis, and mycosis, should be excluded as causes of aortic aneurysms. The progression of these infections can be indolent and, unless they are considered as differential diagnoses, missing them is easy. The clinician should keep in mind that, after the infection is stopped, he or she still may have to treat consequent inflammatory processes.

Other systemic vasculitides and granulomatous diseases, such as giant cell (temporal) arteritis, sarcoidosis, SLE, and Behçet disease, can manifest with aortic lesions. Age younger than 40 years at onset of disease is the single most discriminatory variable between Takayasu arteritis and giant cell (temporal) arteritis.

Workup

Laboratory Studies

  • Elevated acute phase reactants, such as ESR and CRP level, are nonspecific indicators of inflammation. Kerr et al questioned the value of these tests for monitoring the activity of Takayasu arteritis after seeing poor predictive value in relation to the status of surgical specimens.
  • In one study by Eichorn et al4 , high titers of serum anti-endothelial cell antibodies were detected in patients with Takayasu arteritis. The value of this titer in diagnosis and its usefulness as a marker of disease activity have not been completely established.
  • Matrix metalloproteinase (MMP) is elastic proteinase that can degrade elastin in large vessel walls. A variety of inflammatory cytokines are known to induce the enzyme. Matsuyama et al6 reported that plasma level of MMP-9 and MMP-3 were useful markers of activity of Takayasu arteritis.

Imaging Studies

  • Early abnormalities in patients with Takayasu arteritis are limited to the arterial wall. Aortography and arteriography, which had been considered the diagnostic tests for Takayasu arteritis, can demonstrate luminal changes such as stenosis, occlusion, or aneurysmal dilatation, but they are not useful for detecting early mural findings (eg, tree-bark, endothelial wrinkling). Magnetic resonance angiography (MRA) and CT angiography (CTA) may be of similar value. CTA must be performed with a high-resolution (spiral/helical or Ultrafast) CT scanner. MRA and CTA are replacing the conventional aortography as the diagnostic tools of Takayasu arteritis. The recent advance in this field is fluorodeoxyglucose-PET (FDG-PET), which seems to have a role in monitoring the activity of aortitis.
  • CTA and MRA are less invasive than conventional angiography, and they may reveal vascular wall thickening during the early phase of disease. In a study by Yamada et al7 , 25 patients with symptoms suggestive of Takayasu arteritis underwent both conventional angiography and CT helical scanning angiography. CT angiography was 95% sensitive and 100% specific for the diagnosis of Takayasu arteritis, and it was more sensitive than conventional angiography in detecting vessel mural changes. These modalities also can play roles in the follow-up of patients, because MRI and CT scans are able to show reduction of wall thickening after initiation of treatment.
  • In selected patients, conventional arteriography still may be necessary at the time of diagnosis of the late occlusive phase to provide additional information about the degree and extent of the arteritis.
  • Careful interpretation of the chest x-ray also is very important, because some findings, such as widened mediastinum (ie, wide aorta), can be clues to the diagnosis of Takayasu arteritis.
  • Ultrasonographic studies might be useful to follow the diameter and wall thickness changes in specific regions of accessible arterial vessels.
  • A new classification of angiographic findings in patients with Takayasu arteritis was proposed at the International Conference on Takayasu Arteritis, as follows:
    • Type I involves branches of the aortic arch.
    • Type IIa involves the ascending aorta, aortic arch, and its branches.
    • Type IIb involves the type IIa region plus the thoracic descending aorta.
    • Type III involves the thoracic descending aorta, abdominal aorta, and/or renal arteries.
    • Type IV involves only the abdominal aorta and/or renal arteries.
    • Type V involves the whole aorta and its branches.
  • Type V is the most common finding, and type IV is observed in India and Thailand but is very rare in the United States and Japan.
  • Webb et al showed that FDG-PET had high sensitivity (92%) and specificity (100%) for active inflammation in Takayasu arteritis. Andrews et al8 and Meller et al9 concluded that FDG-PET was useful to detect active inflammation of large vessels, and that MRA was able to show progression of vascular wall thickening.

Histologic Findings

The histologic features of arteritis are characterized as focal panarteritis. The intima is markedly thickened by accumulation of mucopolysaccharides. The media and adventitia demonstrate mixed cellular infiltration with granuloma and giant cells. The lesions usually are focal skip lesions rather than the diffuse involvement observed in patients with syphilitic aortitis. See Images 1-2.

Staging

A triphasic pattern of disease progression has been described, as follows:

  • Phase I is the prepulseless inflammatory period characterized by nonspecific systemic symptoms, including low-grade fever, fatigue, arthralgia, and weight loss.
  • Phase II involves vascular inflammation associated with pain (eg, carotidynia) and tenderness over the arteries.
  • Phase III is the fibrotic stage, with predominant ischemic symptoms and signs secondary to dilation, narrowing, or occlusion of the proximal or distal branches of the aorta.

More on Aortitis

Overview: Aortitis
Differential Diagnoses & Workup: Aortitis
Treatment & Medication: Aortitis
Follow-up: Aortitis
Multimedia: Aortitis
References
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References

  1. Rojo-Leyva F, Ratliff NB, Cosgrove DM. Study of 52 patients with idiopathic aortitis from a cohort of 1,204 surgical cases. Arthritis Rheum. Apr 2000;43(4):901-7. [Medline].

  2. Vanoli M, Daina E, Salvarani C, Sabbadini MG, Rossi C, Bacchiani G. Takayasu's arteritis: A study of 104 Italian patients. Arthritis Rheum. Feb 15 2005;53(1):100-7. [Medline].

  3. Hall S, Barr W, Lie JT. Takayasu arteritis. A study of 32 North American patients. Medicine (Baltimore). Mar 1985;64(2):89-99. [Medline].

  4. Eichhorn J, Sima D, Thiele B. Anti-endothelial cell antibodies in Takayasu arteritis. Circulation. Nov 15 1996;94(10):2396-401. [Medline].

  5. Chauhan SK, Tripathy NK, Nityanand S. Antigenic targets and pathogenicity of anti-aortic endothelial cell antibodies in Takayasu arteritis. Arthritis Rheum. Jul 2006;54(7):2326-33. [Medline].

  6. Matsuyama A, Sakai N, Ishigami M. Matrix metalloproteinases as novel disease markers in Takayasu arteritis. Circulation. Sep 23 2003;108(12):1469-73. [Medline].

  7. Yamada I, Nakagawa T, Himeno Y. Takayasu arteritis: evaluation of the thoracic aorta with CT angiography. Radiology. Oct 1998;209(1):103-9. [Medline].

  8. Andrews J, Al-Nahhas A, Pennell DJ. Non-invasive imaging in the diagnosis and management of Takayasu's arteritis. Ann Rheum Dis. Aug 2004;63(8):995-1000. [Medline].

  9. Meller J, Strutz F, Siefker U. Early diagnosis and follow-up of aortitis with [(18)F]FDG PET and MRI. Eur J Nucl Med Mol Imaging. May 2003;30(5):730-6. [Medline].

  10. Hoffman GS, Leavitt RY, Kerr GS. Treatment of glucocorticoid-resistant or relapsing Takayasu arteritis with methotrexate. Arthritis Rheum. Apr 1994;37(4):578-82. [Medline].

  11. Daina E, Schieppati A, Remuzzi G. Mycophenolate mofetil for the treatment of Takayasu arteritis: report of three cases. Ann Intern Med. Mar 2 1999;130(5):422-6. [Medline].

  12. Hoffman GS, Merkel PA, Brasington RD. Anti-tumor necrosis factor therapy in patients with difficult to treat Takayasu arteritis. Arthritis Rheum. Jul 2004;50(7):2296-304. [Medline].

  13. Nuenninghoff DM, Hunder GG, Christianson TJ, McClelland RL, Matteson EL. Incidence and predictors of large-artery complication (aortic aneurysm, aortic dissection, and/or large-artery stenosis) in patients with giant cell arteritis: a population-based study over 50 years. Arthritis Rheum. Dec 2003;48(12):3522-31. [Medline].

  14. Matsuyama A, Sakai N, Ishigami M, Hiraoka H, Yamashita S. Minocycline for the treatment of Takayasu arteritis. Ann Intern Med. Sep 6 2005;143(5):394-5. [Medline].

  15. Liang P, Tan-Ong M, Hoffman GS. Takayasu's arteritis: vascular interventions and outcomes. J Rheumatol. Jan 2004;31(1):102-6. [Medline].

  16. Arend WP, Michel BA, Bloch DA. The American College of Rheumatology 1990 criteria for the classification of Takayasu arteritis. Arthritis Rheum. Aug 1990;33(8):1129-34. [Medline].

  17. Bassa A, Desai DK, Moodley J. Takayasu's disease and pregnancy. Three case studies and a review of the literature. S Afr Med J. Feb 1995;85(2):107-12. [Medline].

  18. Cid MC, Font C, Coll-Vinent B. Large vessel vasculitides. Curr Opin Rheumatol. Jan 1998;10(1):18-28. [Medline].

  19. Dabague J, Reyes PA. Takayasu arteritis in Mexico: a 38-year clinical perspective through literature review. Int J Cardiol. Aug 1996;54 Suppl:S103-9. [Medline].

  20. Di Giacomo V. A case of Takayasu's disease occurred over two hundred years ago. Angiology. Nov 1984;35(11):750-4. [Medline].

  21. Fields CE, Bower TC, Cooper LT, Hoskin T, Noel AA, Panneton JM. Takayasu's arteritis: operative results and influence of disease activity. J Vasc Surg. Jan 2006;43(1):64-71. [Medline].

  22. Grasland A, Pouchot J, Hachulla E, Blétry O, Papo T, Vinceneux P. Typical and atypical Cogan's syndrome: 32 cases and review of the literature. Rheumatology (Oxford). Aug 2004;43(8):1007-15. [Medline].

  23. Hata A, Noda M, Moriwaki R. Angiographic findings of Takayasu arteritis: new classification. Int J Cardiol. Aug 1996;54 Suppl:S155-63. [Medline].

  24. Ishikawa K. Natural history and classification of occlusive thromboaortopathy (Takayasu's disease). Circulation. Jan 1978;57(1):27-35. [Medline].

  25. Ishikawa K. Survival and morbidity after diagnosis of occlusive thromboaortopathy (Takayasu's disease). Am J Cardiol. May 1981;47(5):1026-32. [Medline].

  26. Ishikawa K, Maetani S. Long-term outcome for 120 Japanese patients with Takayasu's disease. Clinical and statistical analyses of related prognostic factors. Circulation. Oct 1994;90(4):1855-60. [Medline].

  27. Kerr GS. Takayasu's arteritis. Rheum Dis Clin North Am. Nov 1995;21(4):1041-58. [Medline].

  28. Kerr GS, Hallahan CW, Giordano J. Takayasu arteritis. Ann Intern Med. Jun 1 1994;120(11):919-29. [Medline].

  29. Lupi-Herrera E, Sanchez-Torres G, Marcushamer J. Takayasu's arteritis. Clinical study of 107 cases. Am Heart J. Jan 1977;93(1):94-103. [Medline].

  30. Maksimowicz-McKinnon K, Clark TM, Hoffman GS. Limitations of therapy and a guarded prognosis in an American cohort of Takayasu arteritis patients. Arthritis Rheum. Mar 2007;56(3):1000-9. [Medline].

  31. Maksimowicz-McKinnon K, Hoffman GS. Takayasu arteritis: what is the long-term prognosis?. Rheum Dis Clin North Am. Nov 2007;33(4):777-86, vi. [Medline].

  32. Matsunaga N, Hayashi K, Sakamoto I. Takayasu arteritis: MR manifestations and diagnosis of acute and chronic phase. J Magn Reson Imaging. Mar-Apr 1998;8(2):406-14. [Medline].

  33. Michel BA, Arend WP, Hunder GG. Clinical differentiation between giant cell (temporal) arteritis and Takayasu's arteritis. J Rheumatol. Jan 1996;23(1):106-11. [Medline].

  34. Misra R, Aggarwal A, Chag M. Raised anticardiolipin antibodies in Takayasu's arteritis. Lancet. Jun 25 1994;343(8913):1644-5. [Medline].

  35. Nakabayashi K, Kurata N, Nangi N. Pulmonary artery involvement as first manifestation in three cases of Takayasu arteritis. Int J Cardiol. Aug 1996;54 Suppl:S177-83. [Medline].

  36. Nesher G, Berkun Y, Mates M, Baras M, Rubinow A, Sonnenblick M. Low-dose aspirin and prevention of cranial ischemic complications in giant cell arteritis. Arthritis Rheum. Apr 2004;50(4):1332-7. [Medline].

  37. Numano F, Kobayashi Y. Takayasu arteritis--beyond pulselessness. Intern Med. Mar 1999;38(3):226-32. [Medline].

  38. Numano F, Okawara M, Inomata H. Takayasu's arteritis. Lancet. Sep 16 2000;356(9234):1023-5. [Medline].

  39. Rao SA, Mandalam KR, Rao VR. Takayasu arteritis: initial and long-term follow-up in 16 patients after percutaneous transluminal angioplasty of the descending thoracic and abdominal aorta. Radiology. Oct 1993;189(1):173-9. [Medline].

  40. Rodriguez-Pla A, de Miguel G, Lopez-Contreras J. Bilateral blindness in Takayasu's disease. Scand J Rheumatol. 1996;25(6):394-5. [Medline].

  41. Seko Y. Takayasu arteritis: insights into immunopathology. Jpn Heart J. Jan 2000;41(1):15-26. [Medline].

  42. Sharma BK, Jain S, Sagar S. Systemic manifestations of Takayasu arteritis: the expanding spectrum. Int J Cardiol. Aug 1996;54 Suppl:S149-54. [Medline].

  43. Subramanyan R, Joy J, Balakrishnan KG. Natural history of aortoarteritis (Takayasu's disease). Circulation. Sep 1989;80(3):429-37. [Medline].

  44. Sun Y, Yip PK, Jeng JS. Ultrasonographic study and long-term follow-up of Takayasu's arteritis. Stroke. Dec 1996;27(12):2178-82. [Medline].

  45. Yasuda K. Surgical treatment of Takayasu's Arteritis. Intern Med. Nov 1998;37(11):903-4. [Medline].

  46. Zhuang H, Yu JQ, Alavi A. Applications of fluorodeoxyglucose-PET imaging in the detection of infection and inflammation and other benign disorders. Radiol Clin North Am. Jan 2005;43(1):121-34. [Medline].

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Further Reading

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Keywords

aortitis, Takayasu's arteritis, Takayasu arteritis, pulseless disease, pulselessness, giant cell arteritis, polyarteritis nodosa, Kawasaki disease, large vessel arteritis, vasculitis

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Contributor Information and Disclosures

Author

Masato Okada, MD, FACP, FACR, FAAAAI, Consulting Staff, Section of Allergy and Rheumatology, Department of Medicine, St Luke's International Hospital, Tokyo
Masato Okada, MD, FACP, FACR, FAAAAI is a member of the following medical societies: American Academy of Allergy Asthma and Immunology, American College of Physicians, and American College of Rheumatology
Disclosure: Nothing to disclose.

Coauthor(s)

Justin D Pearlman, MD, PhD, ME, MA, Director of Dartmouth Advanced Imaging Center, Professor of Medicine, Professor of Radiology, Adjunct Professor, Thayer Bioengineering and Computer Science, Dartmouth-Hitchcock Medical Center
Justin D Pearlman, MD, PhD, ME, MA is a member of the following medical societies: American College of Cardiology, American College of Physicians, American Federation for Medical Research, International Society for Magnetic Resonance in Medicine, and Radiological Society of North America
Disclosure: Nothing to disclose.

Medical Editor

Russell F Kelly, MD, Program Director, Assistant Professor, Department of Internal Medicine, Division of Cardiology, Cook County Hospital, Rush Medical College
Russell F Kelly, MD is a member of the following medical societies: American College of Cardiology
Disclosure: Nothing to disclose.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: Nothing to disclose.

Managing Editor

Ronald J Oudiz, MD, Director of Pulmonary Hypertension, Associate Professor, Department of Medicine, Division of Cardiology, Harbor-UCLA Medical Center, David Geffen School of Medicine at UCLA
Ronald J Oudiz, MD is a member of the following medical societies: American College of Cardiology, American College of Physicians, and American Heart Association
Disclosure: Actelion Grant/research funds Clinical Trials + honoraria; Encysive Grant/research funds Clinical Trials + honoraria; Gilead Grant/research funds Clinical Trials + honoraria; Pfizer Grant/research funds Clinical Trials + honoraria; United Therapeutics Grant/research funds Clinical Trials + honoraria

CME Editor

Amer Suleman, MD, Consultant in Electrophysiology and Cardiovascular Medicine, Department of Internal Medicine, Division of Cardiology, Medical City Dallas Hospital
Amer Suleman, MD is a member of the following medical societies: American College of Physicians, American Heart Association, American Institute of Stress, American Society of Hypertension, Federation of American Societies for Experimental Biology, Royal Society of Medicine, and Society of Cardiac Angiography and Interventions
Disclosure: Nothing to disclose.

Chief Editor

George A Stouffer III, MD, Henry A Foscue Distinguished Professor of Medicine and Cardiology, Director of Interventional Cardiology, Cardiac Catheterization Laboratory, Chief of Clinical Cardiology, Division of Cardiology, University of North Carolina Medical Center
George A Stouffer III, MD is a member of the following medical societies: Alpha Omega Alpha, American College of Cardiology, American College of Physicians, American Heart Association, Phi Beta Kappa, and Society for Cardiac Angiography and Interventions
Disclosure: Nothing to disclose.

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