Aortitis Treatment & Management

Updated: Jul 11, 2019
  • Author: Justin D Pearlman, MD, ME, PhD, FACC, MA; Chief Editor: Richard A Lange, MD, MBA  more...
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Treatment

Medical Care

The primary goals of therapy are to (1) stop progression of inflammatory disease, (2) treat complications, and (3) monitor for reactivation. If vasculitis stems from infection, eradicating the infection prior to initiating immune suppression therapy is generally vital.

The mainstay of therapy for arteritis is corticosteroids; however, a substantial percentage of patients require additional immunosuppressive agents such as cyclophosphamide, methotrexate, or mycophenolate mofetil. [26]

Daily prednisone in doses of 1 mg/kg, not to exceed 60 mg/d, should be given for 1-3 months to patients with active arteritis. When the symptoms and laboratory test results related to the inflammatory process improve, the prednisone should be tapered slowly over several months. The maximum reduction should be 10% of the daily amount per week. Long-term, low-dose prednisone therapy may be necessary to prevent progression of arterial stenoses.

As many as 75% of patients respond favorably to daily prednisone therapy, but the remaining patients whose disease relapses with corticosteroid tapering should receive additional immunosuppressants.

Weekly doses of methotrexate are thought to be less toxic than daily doses of cyclophosphamide. In a study by Hoffman et al of 16 patients whose vascular disease was resistant to corticosteroid therapy, weekly methotrexate (mean dose 17.1 mg; range 10-25 mg) produced remissions in 81%. [27] Relapse occurred in 44% when the 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.

A case report exists of three patients with resistant disease who showed treatment benefit with mycophenolate mofetil (1 g PO bid). [28] The lower toxicity of mycophenolate mofetil makes this regimen an attractive alternative.

Anti-tumor necrosis factor agents (anti-TNFs), etanercept and infliximab, have been administered to patients with active and relapsing Takayasu arteritis despite glucocorticoid therapy. Fourteen of 15 patients responded to therapy and 10 patients achieved sustained remission. [29] Larger and randomized controlled studies are necessary to determine the role of anti-TNFs in the management of aortitis.

Aortic involvement in temporal arteritis (giant cell arteritis) is not uncommon. Nuenninghoff at al reported that 46 of 168 patients (27%) had large-artery complications. [30] Eighteen percent of the patients had aortic aneurysm and/or aortic dissection, and 13% developed large-artery stenosis. Low-dose aspirin in addition to glucocorticoid is accepted as a standard in the initial treatment of giant cell arteritis because of the benefit in reducing the rate of visual loss and cerebrovascular accidents. Long-term, low-dose aspirin has not been shown to have any effect in aortic complication.

Matsuyama et al administered minocycline, 100 mg bid, for 3 months to 11 patients who had active Takayasu arteritis despite prednisolone therapy. [31] Nine of 11 patients were considered to be in remission at the end of the study.

For both Takayasu aortoarteritis and giant cell arteritis with or without aortic involvement, tocilizumab, a humanized monoclonal antibody against the IL-6 receptor, seems to be effective. [32]

No reliable method of determining the activity of arteritis is established, and no single test should be relied upon. As mentioned above, FDG-PET and MMP-3 and MMP-9 serum levels might provide additional information to monitor the activity of aortitis.

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 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 and homocysteine levels should be monitored, especially if the patients require long-term corticosteroid therapy. [33]

Activity

Activity may be limited by claudication (ie, ischemic pain from limb use) or by aortic insufficiency and congestive heart failure.

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Surgical Care

Angioplasty or bypass grafts or stents may be necessary once arterial stenosis has occurred. Unfortunately, according to the experience at the Cleveland Clinic Foundation, vascular interventions for Takayasu arteritis resulted in a high failure rate: 11 of 31 bypass grafts, 3 of 7 percutaneous transluminal angioplasty treatments, and 5 of 7 stents placed experienced restenosis or occlusion. [34]

Surgical repair or angioplasty may be necessary in patients with the following:

  • Significant hypertension resulting from renovascular stenosis

  • Myocardial ischemia secondary to coronary artery involvement

  • Disabling extremity claudication unresponsive to medical treatment

  • Cerebral ischemia

  • Aortic root dilatation leading to significant aortic regurgitation

  • Thoracic or abdominal aortic aneurysms larger than 5 cm in diameter

Stenoses or occlusions affecting lengthy portions of an artery may make angioplasty of an involved segment technically difficult. In addition, the heavily scarred arteries in patients with Takayasu arteritis sometimes are not managed as easily by angioplasty as are atherosclerotic lesions.

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Consultations

Consultations include the following:

  • Infectious disease - To eliminate possibility of Neisseria, Rickettsia, spirochete, fungal, or viral (herpes, hepatis B, hepatitis C) causes

  • Rheumatology - To investigate the many immunologic diseases that may result in vasculitis, including Henoch-Schönlein purpura, SLE, rheumatoid arthritis, cryoglobulinemia, serum sickness, granulomatosis with polyangiitis, Churg-Strauss syndrome, Goodpasture syndrome, and Kawasaki disease

  • Cardiology - To evaluate aortic insufficiency, congestive heart failure, ischemia, and stenoses, for consideration of valve replacement, aneurysm repair, angioplasty, or stent placement

  • Cardiovascular surgery - To evaluate carotid stenosis, aortic dilation, arterial bypasses, and/or perform diagnostic biopsy

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Long-Term Monitoring

Monitor periodically for complications and for progression of the inflammatory processes. Clinical evaluation with careful history review for any new or progressive signs is essential. [1] Periodic examinations should include funduscopic examination, pulse and pressure assessments in all limbs, checking for bruits and signs of abdominal aneurysm, and neurologic examination. No particular blood test has proven reliable, but a variety may be useful if they happen to indicate increased activation.

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Deterrence/Prevention

Aortic trauma with dissection, transplants, immune disorders including connective tissue diseases and inflammatory bowel diseases, infections, and medications that may induce immune complex disease should all raise suspicion for subsequent vasculitis. Vague constitutional symptoms, neck pain, or headaches likewise should raise suspicion for early diagnosis.

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