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Takayasu Arteritis Treatment & Management

  • Author: Jefferson R Roberts, MD; Chief Editor: Herbert S Diamond, MD  more...
 
Updated: Apr 13, 2016
 

Approach Considerations

Medical management of Takayasu arteritis depends on the disease activity and the complications that develop. Some patients have only mild forms of Takayasu arteritis; others deteriorate considerably. The two most important aspects of treatment are controlling the inflammatory process and controlling hypertension.

Corticosteroids

Corticosteroids are the mainstay of therapy for active Takayasu arteritis. However, some patients may also require cytotoxic agents, to achieve remission and taper of long-term corticosteroid treatment.

Oral corticosteroids are started at 1 mg/kg daily or divided twice daily and tapered over weeks to months as symptoms subside.

Long-term, low-dose corticosteroid therapy may be required. Osteoporosis prevention when patients are started on corticosteroids should be seriously considered.

IL-6 receptor inhibitor

The humanized monoclonal antibody tocilizumab been proposed as a treatment for Takayasu arteritis through blockade of the soluble interleukin-6 (IL-6) receptor. This hypothesis arose with emerging evidence supporting IL-6 as a major component in the proinflammatory process of large-vessel vasculitis.

Salvarani et al successfully treated two patients with diagnosed Takayasu arteritis using tocilizumab. Remission was induced in both patients and was achieved in one patient using tocilizumab as monotherapy. Both patients were then transitioned to methotrexate for maintenance therapy and were in clinical remission at follow up 7-10 months later.[36] No large randomized control trials have confirmed this finding, however.

B-cell depletion

Rituximab, a chimeric IgG1 antibody that binds to CD20 expressed on the surface of B cells, has been shown to improve clinical signs and symptoms of Takayasu arteritis.[37] Although the pathogenesis of Takayasu arteritis is not believed to be mediated by the humoral immune system, B cells are believed to have an antibody-independent effect, which may modulate regulatory T-cell immune reactions against foreign and self-antigens. No large trial has proved this finding.

Cytotoxic agents

Cytotoxic agents are used for patients whose disease is steroid resistant or relapsing. These agents are usually continued for at least 1 year after remission and are then tapered to discontinuation. Methotrexate, azathioprine, and cyclophosphamide are among the drugs used in Takayasu arteritis, although success has also been reported with mycophenolate mofetil and tacrolimus.[38] Cyclophosphamide should be reserved for patients with the most severe and refractory disease states.

Anti-tumor necrosis factor agents

Anti–tumor necrosis factor (anti-TNF) agents have shown encouraging results in a small number of patients with relapsing Takayasu arteritis.[39]

In an uncontrolled series of 15 patients, adjunctive treatment with anti-TNF agents was effective in patients with active, relapsing Takayasu arteritis despite treatment with steroids and multiple other immunosuppressive agents.[40] The initial dose of etanercept was 25 mg twice weekly (seven patients); infliximab (11 patients [three were switched from etanercept to infliximab]) was given at 3 mg/kg initially and at 2 weeks, 6 weeks, and then every 8 weeks thereafter. In nine of the 14 responders, an increase in the anti-TNF dosage was required to sustain remission.

The preliminary results of this study suggested that anti-TNF therapy may be a useful adjunct to corticosteroids in the treatment of patients with Takayasu arteritis.

In a review of nine patients with refractory Takayasu arteritis, Youngstein et al reported sustained responses to treatment with a TNF-α antagonist, an IL-6 receptor antagonist, or both. The mean duration of anti–TNF-α treatment was 42 months (maximum 8 years), and two patients maintained responses to IL-6 receptor inhibition at 19 and 20 months.[41]

During treatment, none of the patients showed significant progression in arterial injury, and significant decreases occurred in C-reactive protein level, prednisolone dose, and Indian Takayasu arteritis activity. As five of the nine patients had failed cyclophosphamide, the investigators recommended that therapies targeting TNF-α and the IL-6 receptor be considered ahead of cyclophosphamide.[41]

Cardiovascular procedures

Bypass graft surgery is the procedure with the best long-term patency rate. Percutaneous balloon angioplasty can provide good outcomes for short lesions. Angioplasty and stenting have been used to treat recurrent stenosis. Conventional stents seem to be associated with high failure rates in patients with Takayasu arteritis. Other procedures include aneurysm clipping and revascularization.

Cardiovascular risk factors

Strict management of traditional cardiovascular risk factors such as dyslipidemia, hypertension, and lifestyle factors that increase the risk of cardiovascular disease is mandatory to minimize secondary cardiovascular complications. These complications are the major cause of death in Takayasu arteritis.

Hypertension is treated with antihypertensive agents, and aggressive therapy is necessary to prevent complications. Low-dose aspirin may have a therapeutic effect in large vessel vasculitis.

Antiplatelet agents and heparin may prove useful in preventing stroke. Warfarin also has been used. The literature reports a case of improvement in renal and systemic function with low-dose intravenous (IV) heparin therapy (10,000 U/d) followed by oral anticoagulant and antiplatelet agents.

Pregnancy

Pregnancy is an important concern in Takayasu arteritis; these patients require aggressive treatment. Pregnancy may exacerbate hypertension and/or cardiovascular complications and can increase the risk for maternal and fetal morbidity and mortality. Pregnancy may be safer during presumed remission of Takayasu arteritis. Fetal monitoring is indicated in patients with suspected complications of pregnancy.

Inpatient care

Management of Takayasu arteritis is long-term. Inpatient care is limited to managing acute manifestations of the disease, which usually result in complications from organ failure, stroke, pregnancy, seizures, and intracranial hemorrhage. Intensive care unit (ICU) admission is indicated for patients with critical deterioration.

Diet and activity

Diet modification is necessary to manage hypertension or renal failure. Any activity limitations depend on the severity of the disease and complications.

Consultations

Consult with the following specialists as needed:

  • Rheumatologist
  • Cardiologist
  • Vascular surgeon
  • Imaging/interventional radiologist
  • Obstetrician-gynecologist for pregnant patients
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Surgical Therapy

Critical stenotic lesions should be treated by angioplasty or surgical revascularization during periods of remission. Indications for surgical repair or angioplasty are as follows:

  • Renovascular stenosis causing hypertension
  • Coronary artery stenosis leading to myocardial ischemia
  • Extremity claudication induced by routine activity
  • Cerebral ischemia and/or critical stenosis of 3 or more cerebral vessels
  • Aortic regurgitation
  • Thoracic or abdominal aneurysms larger than 5 cm in diameter
  • Severe coarctation of the aorta

Percutaneous transluminal coronary angioplasty is followed by restenosis at the angioplasty site within 1-2 years in a substantial number of patients.

Bypass graft surgery

Bypass graft surgery is the procedure with the best long-term patency rate. Bypass surgery has been performed on patients with critical thoracic aortic arch arterial stenosis, upper and lower extremity ischemia, cerebrovascular accidents, and renal artery stenosis. The procedures are generally case specific. Certain issues, such as the timing of surgery in relation to disease activity or the advisability of surgery in symptom-free patients, have not been resolved. Anastomotic stenoses or graft occlusion is a potential complication of surgery.

Grafts have been used to bypass regions of severe stenosis or occlusion. Usually, the graft is a saphenous vein graft. Examples of grafts performed include bypass of renal artery stenosis for renal salvage; bypass of innominate or carotid artery; and bypass between subclavian-axillary and common carotid arteries. Extraintracranial bypass operations generally are performed for stenosis of the internal carotid or middle cerebral arteries.

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Follow-Up

best long-term patency rate. Bypass surgery has been performed on patients with critical thoracic aortic arch arterial stenosis, upper and lower extremity ischemia, cerebrovascular accidents, and renal artery stenosis. The procedures are generally case specific. Certain issues, such as the timing of surgery in relation to disease activity or the advisability of surgery in symptom-free patients, have not been resolved. Anastomotic stenoses or graft occlusion is a potential complication of surgery.

Grafts have been used to bypass regions of severe stenosis or occlusion. Usually, the graft is a saphenous vein graft. Examples of grafts performed include bypass of renal artery stenosis for renal salvage; bypass of innominate or carotid artery; and bypass between subclavian-axillary and common carotid arteries. Extraintracranial bypass operations generally are performed for stenosis of the internal carotid or middle cerebral arteries.

Follow-up should be with a rheumatologist who can follow disease activity and treat the patient medically as needed.

A follow-up examination of critical or near-critical stenosis and disease activity with angiography (or MRI or CT angiography) and possibly FDG-PET scanning may be necessary. Recognizing that Takayasu arteritis may progress in the absence of clinical findings is important. Periodic imaging may reveal an active disease that requires treatment with immunosuppressive agents.

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Contributor Information and Disclosures
Author

Jefferson R Roberts, MD Chief of Rheumatology Service, Tripler Army Medical Center; Assistant Clinical Professor of Medicine, Uniformed Services University of the Health Sciences

Jefferson R Roberts, MD is a member of the following medical societies: American College of Physicians, American College of Rheumatology, Society for Simulation in Healthcare

Disclosure: Nothing to disclose.

Coauthor(s)

Rodger Stitt, MD Department of Internal Medicine, Tripler Army Medical Center, Honolulu

Rodger Stitt, MD is a member of the following medical societies: American College of Physicians

Disclosure: Nothing to disclose.

 

Disclosure: Nothing to disclose.

Phalgoon A Shah, MD Resident Physician, Department of Medicine, Tripler Army Medical Center

Phalgoon A Shah, MD is a member of the following medical societies: American College of Physicians

Disclosure: Nothing to disclose.

Chief Editor

Herbert S Diamond, MD Visiting Professor of Medicine, Division of Rheumatology, State University of New York Downstate Medical Center; Chairman Emeritus, Department of Internal Medicine, Western Pennsylvania Hospital

Herbert S Diamond, MD is a member of the following medical societies: Alpha Omega Alpha, American College of Physicians, American College of Rheumatology, American Medical Association, Phi Beta Kappa

Disclosure: Nothing to disclose.

Additional Contributors

Gabriel Bucurescu, MD, MS Staff Neurologist, Neurology Service, Philadelphia Veterans Affairs Medical Center

Gabriel Bucurescu, MD, MS is a member of the following medical societies: American Academy of Neurology, American Clinical Neurophysiology Society, American Epilepsy Society

Disclosure: Nothing to disclose.

Robert E Wolf, MD, PhD Professor Emeritus, Department of Medicine, Louisiana State University School of Medicine in Shreveport; Chief, Rheumatology Section, Medical Service, Overton Brooks Veterans Affairs Medical Center

Robert E Wolf, MD, PhD is a member of the following medical societies: American College of Rheumatology, Arthritis Foundation, Society for Leukocyte Biology

Disclosure: Nothing to disclose.

Mohammed Mubashir Ahmed, MD Associate Professor, Department of Medicine, Division of Rheumatology, University of Toledo College of Medicine

Mohammed Mubashir Ahmed, MD is a member of the following medical societies: American College of Physicians, American College of Rheumatology, American Federation for Medical Research

Disclosure: Nothing to disclose.

Acknowledgements

Elliot Goldberg, MD Dean of the Western Pennsylvania Clinical Campus, Professor, Department of Medicine, Temple University School of Medicine

Elliot Goldberg, MD is a member of the following medical societies: Alpha Omega Alpha, American College of Physicians, and American College of Rheumatology

Disclosure: Nothing to disclose.

B Mark Keegan, MD, FRCPC Assistant Professor of Neurology, College of Medicine, Mayo Clinic; Master's Faculty, Mayo Graduate School; Consultant, Department of Neurology, Mayo Clinic, Rochester

B Mark Keegan, MD, FRCPC is a member of the following medical societies: American Academy of Neurology, American Medical Association, and Minnesota Medical Association

Disclosure: Novartis Consulting fee Consulting

Sydney Louis, MBBCh, MD Emeritus Professor, Department of Neurology, The Warren Alpert Medical School of Brown University

Sydney Louis, MBBCh, MD is a member of the following medical societies: American Academy of Neurology

Disclosure: Nothing to disclose.

Michael G Rossman, MD, LTC, MC, FS Fellow, Department of Rheumatology, Walter Reed Army Medical Center

Michael G Rossman, MD, LTC, MC, FS is a member of the following medical societies: American College of Physicians, American College of Rheumatology, American Medical Association, and Society of US Army Flight Surgeons

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD Senior Pharmacy Editor, eMedicine

Disclosure: eMedicine Salary Employment

Florian P Thomas, MD, MA, PhD, Drmed Director, Spinal Cord Injury Unit, St Louis Veterans Affairs Medical Center; Director, National MS Society Multiple Sclerosis Center; Director, Neuropathy Association Center of Excellence, Professor, Department of Neurology and Psychiatry, Associate Professor, Institute for Molecular Virology, and Department of Molecular Microbiology and Immunology, St Louis University School of Medicine

Florian P Thomas, MD, MA, PhD, Drmed is a member of the following medical societies: American Academy of Neurology, American Neurological Association, American Paraplegia Society, Consortium of Multiple Sclerosis Centers, and National Multiple Sclerosis Society

Disclosure: Nothing to disclose.

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Complete occlusion of the left common carotid artery in a 48-year-old woman with Takayasu disease. Also note narrowing of the origin of the right subclavian artery and a narrowed small vessel with subsequent aneurysmal dilatation on the right side. Image courtesy of Robert Cirillo, MD.
Characteristic long, tapered narrowing of the distal aorta and iliac vessels. Image courtesy of Robert Cirillo, MD.
Image obtained in the same patient as in Image 2 reveals narrowing of the proximal descending aorta and right brachiocephalic artery. Image courtesy of Robert Cirillo, MD.
Aortogram of a 15-year-old girl with Takayasu arteritis. Note large aneurysms of descending aorta and dilatation of innominate artery. Image courtesy of Christine Hom, MD.
MRI of thorax of 15-year-old girl with Takayasu arteritis. Note aneurysms of descending aorta. Image courtesy of Christine Hom, MD.
Coronal MRI of abdomen of 15-year-old girl with Takayasu arteritis. Note thickening and tortuosity of abdominal aorta proximal to kidneys. Image courtesy of Christine Hom, MD.
 
 
 
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