Sections

Aortitis

Medication

Medication Summary

No reliable method exists for determining the activity of arteritis. According to sequential angiographic studies and vascular specimens obtained at the time of surgery, active vasculitis is present in approximately 50% of patients who lack symptoms of active inflammation or have a normal ESR. To prevent progression of vascular lesions and to reduce the necessity of surgical procedures in the later stage, careful monitoring of disease activity with sequential imaging studies and more prolonged immunosuppressive treatments may be necessary.

As the prognosis of patients with Takayasu arteritis improves, prevention of atherosclerotic disorders becomes more important. Treatment of hypertension and congestive heart failure should be instituted if these complications occur, and serum cholesterol levels should be monitored, especially if the patients require long-term corticosteroid therapy.

Class Summary

These agents have anti-inflammatory properties and cause profound and varied metabolic effects. They modify the body's immune response to diverse stimuli.

Prednisone (Deltasone, Orasone, Sterapred)

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Mainstay of therapy. May decrease inflammation by reducing capillary permeability and suppressing leukocyte activity.

Daily doses should be given to patients with active Takayasu arteritis. As many as 75% of patients respond favorably to this regimen, but remaining patients, and patients who relapse with tapering, must receive additional immunosuppressants. When tapering steroids, maximum reduction should be 10% of daily amount per week. Long-term low-dose therapy may be necessary to prevent progression of arterial stenoses. Complications of corticosteroid therapy include aseptic necrosis of hip, corticosteroid dependence, and gastric ulcers.

Class Summary

A substantial percentage of patients with aortitis or other forms of vasculitis require additional immunosuppressive agents (eg, cyclophosphamide, methotrexate, mycophenolate mofetil).

Methotrexate (Folex PFS, Rheumatrex)

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The mechanism of action in treatment of inflammatory reactions is unknown. May affect immune function. Ameliorates symptoms of inflammation (eg, pain, swelling, stiffness).

Weekly doses of cyclophosphamide are thought to be less toxic than daily doses. In one study of 16 patients whose disease was resistant to corticosteroid therapy, weekly methotrexate (mean dose 17.1 mg; range 10-25 mg) induced remissions in 81%. Relapse occurred in 44% when corticosteroids were tapered to or near discontinuation. Reinstitution of corticosteroids led to remission, and 3 of 7 patients in this group successfully stopped glucocorticoid therapy.

Mycophenolate (CellCept)

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Inhibits purine synthesis and proliferation of human lymphocytes. Reduced toxicity makes this regimen an attractive alternative.

Cyclophosphamide (Cytoxan)

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Chemically related to nitrogen mustards. As alkylating agent, mechanism of action of active metabolites may involve cross-linking of DNA, which may interfere with growth of normal and neoplastic cells.

Class Summary

These agents are disease modifying drugs.

Etanercept (Enbrel)

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Soluble p75 TNF receptor fusion protein (sTNFR-Ig). Inhibits TNF binding to cell surface receptors, which decreases inflammatory and immune responses.

Infliximab (Remicade)

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Chimeric IgG1k monoclonal antibody that neutralizes cytokine TNF-a and inhibits its binding to TNF-a receptor. Reduces infiltration of inflammatory cells and TNF-a production in inflamed areas. Used with methotrexate in patients who have had inadequate response to methotrexate monotherapy.

Class Summary

Empiric antimicrobial therapy should cover all likely pathogens in the context of the clinical setting.

Minocycline (Dynacin, Minocin)

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Treats infections caused by susceptible gram-negative and gram-positive organisms, in addition to infections caused by susceptible chlamydia, rickettsia, and mycoplasma. Additionally, has anti-inflammatory properties.

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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. 2003 May. 30(5):730-6. [QxMD MEDLINE Link].
Johnston SL, Lock RJ, Gompels MM. Takayasu arteritis: a review. J Clin Pathol. 2002 Jul. 55(7):481-6. [QxMD MEDLINE Link]. [Full Text].
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Daina E, Schieppati A, Remuzzi G. Mycophenolate mofetil for the treatment of Takayasu arteritis: report of three cases. Ann Intern Med. 1999 Mar 2. 130(5):422-6. [QxMD MEDLINE Link].
Hoffman GS, Merkel PA, Brasington RD. Anti-tumor necrosis factor therapy in patients with difficult to treat Takayasu arteritis. Arthritis Rheum. 2004 Jul. 50(7):2296-304. [QxMD MEDLINE Link].
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. 2003 Dec. 48(12):3522-31. [QxMD MEDLINE Link].
Matsuyama A, Sakai N, Ishigami M, Hiraoka H, Yamashita S. Minocycline for the treatment of Takayasu arteritis. Ann Intern Med. 2005 Sep 6. 143(5):394-5. [QxMD MEDLINE Link].
Unizony S, Stone JH, Stone JR. New treatment strategies in large-vessel vasculitis. Curr Opin Rheumatol. 2013 Jan. 25(1):3-9. [QxMD MEDLINE Link].
Bruls S, Courtois A, Nusgens B, et al. 18F-FDG PET/CT in the management of aortitis. Clin Nucl Med. 2016 Jan. 41(1):28-33. [QxMD MEDLINE Link].
Liang P, Tan-Ong M, Hoffman GS. Takayasu's arteritis: vascular interventions and outcomes. J Rheumatol. 2004 Jan. 31(1):102-6. [QxMD MEDLINE Link].
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Espitia O, Samson M, Le Gallou T, et al. Comparison of idiopathic (isolated) aortitis and giant cell arteritis-related aortitis. A French retrospective multicenter study of 117 patients. Autoimmun Rev. 2016 Jun. 15(6):571-6. [QxMD MEDLINE Link].
Kassar K, Lucke M, Pu C, Tsukashita M. Fulminant infectious aortitis with ascending aortic rupture. Ann Thorac Surg. 2017 Jan. 103(1):e11-e12. [QxMD MEDLINE Link].
Williams ZB, Ryden LE, Organ NM. Aortitis causing rapid growth of a mycotic aortic aneurysm. J Surg Case Rep. 2016 Mar 22. 2016(3):[QxMD MEDLINE Link]. [Full Text].
Silvestri V, Isernia G. Suspected giant cell aortitis : from multiple aortic structural damage to fatal listeria sepsis, a case report. Ann Vasc Surg. 2017 Jul. 42:307.e1-307.e6. [QxMD MEDLINE Link].
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Cha MJ, Chong S, Kim YS, Park B, Seo JH, Lee ES. Immunoglobulin G4-related periaortitis involving the aortic arch mimicking a mediastinal tumor. Ann Thorac Surg. 2017 Mar. 103(3):e267-e270. [QxMD MEDLINE Link].
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Media Gallery

Aortitis. This chart represents the presence of an associated morbidity in Takayasu arteritis in the United States (adapted from combined reports by Maksimowicz-McKinnon et al and Kerr).
Aortitis. The frequency of vascular involvement in Takayasu arteritis is depicted (adapted from combined reports by Maksimowicz-McKinnon et al and Kerr).
Aortitis. Diffuse stenosis from the aortic arch to the abdominal aorta. The left common carotid artery is also stenotic (top arrow) and the left subclavian artery is not visualized (second arrow from the top).
Aortitis. Bilateral dilatation of the vertebral arteries. Occlusion of the right internal carotid artery (left arrow). Severe stenosis of the left internal carotid artery are shown. Note the moderate stenosis of the left external carotid artery at the bifurcation with dilated collateral circulation.
Aortitis. This image reveals moderate stenosis of the external iliac artery at the bifurcation and occlusion of the right femoral artery.
Aortitis. This image demonstrates leukocyte infiltration of the vasa vasorum of the aorta accompanies arteritis obliterans and ischemic necrosis of the media in a case of syphilitic aortitis.
Aortitis. Granulomatous arteritis with thrombosis of a cerebral vessel may present as a neurologic defect with no obvious vascular disease by history or physical examination.

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Author

Justin D Pearlman, MD, ME, PhD, FACC, MA Chief, Division of Cardiology, Director of Cardiology Consultative Service, Director of Cardiology Clinic Service, Director of Cardiology Non-Invasive Laboratory, Chair of Institutional Review Board, University of California, Los Angeles, David Geffen School of Medicine

Justin D Pearlman, MD, ME, PhD, FACC, MA is a member of the following medical societies: American College of Cardiology, International Society for Magnetic Resonance in Medicine, American College of Physicians, American Federation for Medical Research, Radiological Society of North America

Disclosure: Nothing to disclose.

Coauthor(s)

Masato Okada, MD, FACP, FACR, FAAAAI Chief, Immuno-Rheumatology Center, St Luke's International Hospital, Japan

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, American College of Rheumatology

Disclosure: Nothing to disclose.

Specialty Editor Board

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Received salary from Medscape for employment. for: Medscape.

Ronald J Oudiz, MD, FACP, FACC, FCCP Professor of Medicine, University of California, Los Angeles, David Geffen School of Medicine; Director, Liu Center for Pulmonary Hypertension, Division of Cardiology, LA Biomedical Research Institute at Harbor-UCLA Medical Center

Ronald J Oudiz, MD, FACP, FACC, FCCP is a member of the following medical societies: American College of Cardiology, American College of Chest Physicians, American College of Physicians, American Heart Association, American Thoracic Society

Disclosure: Serve(d) as a speaker or a member of a speakers bureau for: Actelion, Bayer, Gilead, United Therapeutics<br/>Received research grant from: Actelion, Arena, Gilead, GSK, Liquidia, Reata, United Therapeutics<br/>Received income in an amount equal to or greater than $250 from: Actelion, Complexa, Gilead, Medtronic, Reata, United Therapeutics.

Chief Editor

Richard A Lange, MD, MBA President, Texas Tech University Health Sciences Center, Dean, Paul L Foster School of Medicine

Richard A Lange, MD, MBA is a member of the following medical societies: Alpha Omega Alpha, American College of Cardiology, American Heart Association, Association of Subspecialty Professors

Disclosure: Nothing to disclose.

Additional Contributors

Russell F Kelly, MD Assistant Professor, Department of Internal Medicine, Rush Medical College; Chairman of Adult Cardiology and Director of the Fellowship Program, Cook County Hospital

Russell F Kelly, MD is a member of the following medical societies: American College of Cardiology

Disclosure: Nothing to disclose.

Aortitis Medication

Medication Summary

Corticosteroids

Class Summary

Prednisone (Deltasone, Orasone, Sterapred)

Prednisone (Deltasone, Orasone, Sterapred)

Immunosuppressants