eMedicine Specialties > Rheumatology > Vasculitis

Takayasu Arteritis

Mohammed Mubashir Ahmed, MD, Associate Professor, Department of Medicine, Division of Rheumatology, University of Toledo College of Medicine
Robert E Wolf, MD, PhD, Professor Emeritus, Department of Medicine, Louisiana State University Health Sciences Center at Shreveport; Chief, Rheumatology Section, Medical Service, Overton Brooks Veterans Administration Medical Center of Shreveport

Updated: Feb 14, 2008

Introduction

Background

Takayasu arteritis (TA) is classically defined as a chronic, progressive, inflammatory, occlusive disease of the aorta and its branches. The pulmonary arteries can also be involved. Takayasu arteritis is a systemic disease that may have isolated, atypical, and catastrophic manifestations. Takayasu described the retinal changes of the disease in 1908, the same year that the association between the retinal changes and pulse deficit was reported. However, the disorder was not termed Takayasu's disease until 1954.

Pathophysiology

Cell-mediated autoimmunity appears to play an important role in the mechanism of vascular injury. Takayasu arteritis is characterized by a specific pattern of histopathological changes. The early stage consists of a continuous or patchy granulomatous inflammatory reaction involving macrophages, lymphocytes, and multinucleated giant cells. The infiltrating T lymphocytes may be of restricted phenotype and have a restricted repertoire, suggesting an antigenic target in aortic tissue. Antiendothelial antibodies may be found in high titers in some patients, but this may be an epiphenomenon rather than one of pathogenetic importance.

Frequency

United States

The estimated annual prevalence is 2.6-6.4 persons per 1,000,000 population. The discrepancy is attributed to genetic factors and difficulty in diagnosis. Between 1971 and 1983 in Olmstead County, Minnesota, 3 cases were recorded, thus giving an annual incidence of 2.6 cases per million population.

International

Although Takayasu arteritis has a worldwide distribution, it is observed more frequently in Asia and India than in Western Europe and North America. Worldwide incidence is estimated at 2.6 cases per million persons per year. The prevalence in Sweden is similar to that in the United States (ie, 2.6-6.4 persons per million population). In the United Kingdom, the annual incidence is 0.15 cases per million persons.

Mortality/Morbidity

Takayasu arteritis is a chronic relapsing and remitting disorder. The 15-year survival rate has been reported to be 90-95%. In other studies, the mortality rate has been reported to range from 2-35% over 5 years. Such disparity may reflect differences in access to care, definitions of disease activity, and indications for treatment.

The overall morbidity depends on the severity of the lesions and their consequences and is usually the result of vascular complications such as aortic regurgitation, congestive heart failure, cerebrovascular events, myocardial infarction, aneurysm rupture, or renal failure.

Race

Takayasu arteritis is observed more frequently in patients of Asian or Indian descent. Japanese patients with Takayasu arteritis have a higher incidence of aortic arch involvement. In contrast, series from India report higher incidences of thoracic and abdominal involvement. In US patients with Takayasu arteritis, the most commonly involved vessels are the left subclavian, superior mesenteric, and abdominal aorta.

Sex

Approximately 80% of patients are women.

Age

Most patients are aged 4-63 years, with the mean age of onset being approximately 30 years. Fewer than 15% of cases present in individuals older than 40 years.

Clinical

History

The presentation of Takayasu arteritis is heterogeneous. Most patients present with systemic and vascular symptoms; however, approximately 20% of patients with Takayasu arteritis are clinically asymptomatic, with the disease being detected based on abnormal vascular findings upon examination. Constitutional symptoms usually precede clinical vascular involvement.

• The American College of Rheumatology has established classification criteria for Takayasu arteritis (3 of 6 criteria are necessary), as follows:
  • Age of 40 years or younger at disease onset
  • Claudication of the extremities
  • Decreased pulsation of one or both brachial arteries
  • Difference of at least 10 mm Hg in systolic blood pressure between arms
  • Bruit over one or both subclavian arteries or the abdominal aorta
  • Arteriographic narrowing or occlusion of the entire aorta, its primary branches, or large arteries in the upper or lower extremities that is not due to arteriosclerosis, fibromuscular dysplasia, or other causes
• Constitutional symptoms are typical in 40% of North American patients. They include the following:
  • Fever (40%; some patients initially present with fever of unknown origin in the prepulseless phase)
  • Weight loss (40-60%)
  • Malaise (40-60%)
  • Fatigue (40%)
  • Myalgias (40%)
  • Arthralgias (40-60%)
  • Arthritis (40%)
• Vascular features (60-70%) include the following:
  • Jaw claudication
  • Extremity claudication
• Neurological features (60%) include the following:
  • Transient ischemic attacks
  • Hemorrhagic or ischemic stroke
  • Transient or permanent blindness
  • Headaches
  • Seizures
  • Subclavian steal syndrome
• Cardiac features (50%) include the following:
  • Aortic regurgitation
  • Angina
  • Myocardial infarction
  • Myocarditis and cardiomyopathy
  • Congestive heart failure (primary cause of death)
  • Arrhythmias
  • Sudden death
• Pulmonary features (40%) include the following:
  • Pulmonary hypertension (often asymptomatic)
  • Hemoptysis
  • Pleuritis
  • Compromised pulmonary function tests
  • Abnormal findings on ventilation/perfusion scans (may be mistakenly diagnosed as thromboembolic disease)
• Renal manifestations include the following:
  • Renovascular hypertension (most frequent)
  • Mesangial proliferative glomerulonephritis with mesangial deposits of immunoglobulins M, G, and A and C3
  • Membranoproliferative and crescentic glomerulonephritis
  • Renal amyloidosis (rarely reported)
• Dermatological manifestations include the following:
  • Ulcerated subacute nodular lesions
  • Erythema nodosum
  • Erythema induratum
  • Papulonecrotic eruptions
  • Papular erythematous lesions of hands
  • Pyoderma gangrenosum
  • Erythema multiforme
• Gastrointestinal manifestations are rare and include the following:
  • Nausea
  • Vomiting
  • Diarrhea
  • Abdominal pain
  • Bleeding (due to mesenteric artery ischemia)

Physical

A thorough physical examination is essential, with particular attention to peripheral pulses, blood pressure in all 4 extremities, and an ophthalmologic examination.

• The most discriminatory finding is a systolic blood pressure difference (>10 mm Hg) between arms. Hypertension due to renal artery involvement is found in approximately 50% of patients.
• Absent or diminished pulses are the clinical hallmark of Takayasu arteritis, but pulses are normal in many patients and upper limbs are affected more often than lower limbs.
• Vessel tenderness (carotidynia) may be present.
• Bruits often are noted.
• Aortic regurgitation may be noted upon cardiac examination.
• Of note upon ophthalmologic examination may be retinal hemorrhages, cotton-wool exudates, venous dilatation and beading, microaneurysms of peripheral retina, optic atrophy, vitreous hemorrhage, and classic wreathlike peripapillary arteriovenous anastomoses (extremely rare).
• Skin vasculitis resembling erythema nodosum or ulcerating nodular lesions may be seen.

Causes

Takayasu arteritis is believed to be an autoimmune disease with no known cause. Genetic factors may play a role in the pathogenesis of Takayasu arteritis, with a possible major histocompatibility complex linkage. In Japan and Korea, Takayasu arteritis is associated with human leukocyte antigens HLA-A10, B5, Bw52, DR2, and DR4. These associations have not been confirmed in Western studies. Takayasu arteritis is associated with HLA-B22 in the United States.

A recent study demonstrated an association between 4 unrelated cases of Takayasu arteritis and CD36 deficiency (CD36d).¹ The human CD36 antigen is a multifunctional membrane glycoprotein that belongs to the class B scavenger receptor family. It is expressed on monocytes, platelets, and endothelial cells and contributes to myocardial fatty acid transport. In patients with CD36d, myocardial I-15-(p-iodophenyl)-3-(R,S)-methyl pentadecanoic acid (BMIPP) uptake was absent. CD36 thrombospondin signal is important in the apoptotic regulation of vascular endothelial cells. The defects in apoptotic machinery in patients with CD36d are hypothesized to predispose them to autoimmune disorders such as Takayasu arteritis.

Differential Diagnoses

Other Problems to Be Considered

Fibromuscular dysplasia
Spondyloarthropathies
Thromboembolism
Traumatic stenosis
Radiation fibrosis
Ergotism
Ehlers-Danlos syndrome
Marfan syndrome
Neurofibromatosis
Syphilis
Cogan syndrome

Workup

Laboratory Studies

• The acute phase reactants and clinical parameters generally used to define active inflammatory disease do not universally reflect active blood vessel inflammation in Takayasu arteritis. The erythrocyte sedimentation rate is elevated in most but not all patients during active inflammatory disease. However, it is not a consistently reliable marker of disease activity, with both false-positive and false-negative associations being reported.
• Other markers, including endothelin-1, von Willebrand factor, factor VIII antigen, and thrombomodulin, have been found to be inadequate markers of disease activity.

Imaging Studies

• While imaging studies (CT scan, MRI) show typical patterns of stenoses or aneurysms of the arteries, angiography remains the criterion standard for diagnosis and evaluation of the extent of disease. However, recent studies suggest that noninvasive imaging modalities such as MRI, ultrasonography, and 18F-fluorodeoxyglucose positron emission tomography (18F-FDG-PET) allow diagnosis of Takayasu arteritis earlier in the disease course than standard angiography and provide a means for monitoring disease activity.
  • Angiography can evaluate only the appearance of the lumen and cannot differentiate between active and inactive lesions.
  • Takayasu arteritis can be divided into 6 types based on angiographic involvement.
    • Type I - Branches of the aortic arch
    • Type IIa - Ascending aorta, aortic arch, and its branches
    • Type IIb - Type IIa region plus thoracic descending aorta
    • Type III - Thoracic descending aorta, abdominal aorta, renal arteries, or a combination
    • Type IV - Abdominal aorta, renal arteries, or both
    • Type V - Entire aorta and its branches
• Other tests
  • Magnetic resonance angiography (MRA) is equally or more sensitive than angiography for revealing lesions in the aorta and its brachycephalic branches but is less sensitive for helping detect smaller branch involvement. MRA that uses fast spin-echo sequences designed to enhance the detection of vessel wall edema has shown promise in assessing disease activity before irreversible lesions develop.
  • CT helical scanning angiography also has been shown to be sensitive and specific as a diagnostic tool.
  • Color Doppler ultrasonography provides details of the vascular wall, lumen, and flow and is a useful tool for screening and follow-up, particularly for carotid and subclavian arteries.
  • Positron emission tomography (PET) scanning with radioactive-labeled 18-fluorodeoxyglucose (FDG) has been shown to be useful in monitoring disease activity and response to treatment in preliminary studies. Presence or absence of FDG uptake correlates well with clinical state and MRI findings. Its use in patients with Takayasu arteritis requires further investigation.
  • MRA or CT angiography and FDG-PET may be useful to monitor vascular response to treatment.

Procedures

• Biopsy of medium- to large-sized vessels may be diagnostic in early stages of the disease; however, in the chronic phase, diagnosis by biopsy alone is inadequate. In contrast to other vasculitides, tissue biopsies play little to no role in the diagnosis of Takayasu arteritis, as histologic examination of the great vessels is usually possible only at the time of vascular procedures or postmortem. Whenever possible, the feasibility of submitting some arterial tissue should be discussed with the attending surgeon prior to any surgical revascularization procedure.
• Percutaneous balloon angioplasty can provide good outcomes for short lesions.
• Conventional stents seem to be associated with high failure rate in patients with Takayasu arteritis.
• Bypass graft surgery is the procedure with the best long-term patency rate.

Histologic Findings

Takayasu arteritis is characterized by a special pattern of histopathological changes. The early stage consists of a continuous or patchy granulomatous inflammatory reaction involving macrophages, lymphocytes, and multinucleated giant cells. Inflammation initially occurs in the vasa vasorum, with the artery wall becoming irregularly thickened and the lumen becoming narrowed. Takayasu arteritis progresses to a sclerotic stage, with intimal and adventitial fibrosis and scarring of the media.

Treatment

Medical Care

Therapeutic intervention includes corticosteroids with or without cytotoxic agents.

• Corticosteroids are the mainstay of therapy for active Takayasu arteritis, and some patients may require additional cytotoxic agents to achieve remission and discontinue steroid treatment.
  • Corticosteroids are started at 1 mg/kg/d PO or divided bid/qid 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.
• Cytotoxic agents are used for patients with steroid resistance or relapsing Takayasu arteritis. These agents are usually continued for one year after remission and are then tapered to discontinuation. Agents and doses are as follows:
  • Azathioprine 1-2 mg/kg/d PO
  • Methotrexate 7.5-25 mg/wk PO or IM
  • Cyclophosphamide 2 mg/kg/d PO (should be reserved for patients with the most severe and refractory disease states)
• Cyclosporin A also has been used in steroid-resistant patients at initial doses of 5 mg/kg/d and then 2-3 mg/kg/d for maintenance.
• Mycophenolate mofetil (2 g/d PO) has been used in patients with Takayasu arteritis resistant to steroids and other immunosuppressant drugs.
• In an uncontrolled series of 15 patients, adjunctive treatment with anti–tumor necrosis factor (TNF) agents was effective in patients with active, relapsing Takayasu arteritis despite treatment with steroids and multiple other immunosuppressive agents.² The initial dose of etanercept was 25 mg twice weekly (7 patients), and infliximab (11 patients [3 were switched from etanercept to infliximab]) was given at 3 mg/kg initially and at 2 weeks, 6 weeks, and every 8 weeks thereafter. In 9 of the 14 responders, an increase in the anti-TNF dosage was required to sustain remission. These preliminary results suggest that anti-TNF therapy may be a useful adjunct to corticosteroids in the treatment of patients with Takayasu arteritis and require further studies. A larger randomized controlled study of anti-TNF therapy for Takayasu arteritis is planned.
• Strict management of traditional cardiovascular risk factors such as dyslipidemia, hypertension, and lifestyle factors is mandatory to minimize secondary cardiovascular complications, which are the major cause of death in this disease. Additionally, low-dose aspirin may have a therapeutic effect in large vessel vasculitis.

Surgical Care

• 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 procedures have the best long-term patency rate.

Consultations

• Rheumatologist
• Cardiologist
• Vascular surgeon
• Imaging/interventional radiologist

Medication

The goals of pharmacotherapy are to reduce morbidity and to prevent complications.

Immunosuppressive agents

These agents inhibit key factors that mediate immune reactions.

Prednisone (Deltasone, Orasone, Meticorten)

Corticosteroid, first-line therapy; immunosuppressant for treatment of autoimmune disorders. May decrease inflammation by reversing increased capillary permeability and suppressing PMN activity and CD4 counts.

Dosing

Adult

1 mg/kg/d PO qd or divided bid/qid; taper over 2 wk as symptoms resolve

Pediatric

0.05-2 mg/kg PO divided bid/qid; taper over 2 wk as symptoms resolve

Interactions

Coadministration with estrogens may decrease prednisone clearance; concurrent use with digoxin may cause digitalis toxicity secondary to hypokalemia; phenobarbital, phenytoin, and rifampin may increase metabolism of glucocorticoids (consider increasing maintenance dose); monitor for hypokalemia with coadministration of diuretics

Contraindications

Documented hypersensitivity, viral infection, peptic ulcer disease, hepatic dysfunction, connective tissue infections, fungal or tubercular skin infections, GI disease

Precautions

Pregnancy

B - Fetal risk not confirmed in studies in humans but has been shown in some studies in animals

Precautions

Abrupt discontinuation of glucocorticoids may cause adrenal crisis; hyperglycemia, edema, osteonecrosis, myopathy, peptic ulcer disease, hypokalemia, osteoporosis, euphoria, psychosis, growth suppression, and infections may occur with glucocorticoid use; osteoporosis screening and prevention measures should be instituted when corticosteroid therapy is begun

Cyclophosphamide (Cytoxan, Neosar)

May be added to steroid if minimal response to steroid or if on steroid for prolonged period of time. Chemically related to nitrogen mustards. As an alkylating agent, the mechanism of action of the active metabolites may involve cross-linking of DNA, which may interfere with growth of normal and neoplastic cells.

Dosing

Adult

2 mg/kg PO qd

Pediatric

Not established

Interactions

Allopurinol may increase risk of bleeding or infection and enhance myelosuppressive effects of cyclophosphamide; may potentiate doxorubicin-induced cardiotoxicity; may reduce digoxin serum levels and antimicrobial effects of quinolones
Chloramphenicol may increase half-life of cyclophosphamide while decreasing metabolite concentrations; may increase effect of anticoagulants; coadministration with high doses of phenobarbital may increase rate of metabolism and leukopenic activity of cyclophosphamide; thiazide diuretics may prolong cyclophosphamide-induced leukopenia and neuromuscular blockade by inhibiting cholinesterase activity

Contraindications

Documented hypersensitivity; severely depressed bone marrow function

Precautions

Pregnancy

D - Fetal risk shown in humans; use only if benefits outweigh risk to fetus

Precautions

May cause sterility; regularly examine hematologic profile (particularly neutrophils and platelets) to monitor for hematopoietic suppression; regularly examine urine for RBCs, which may precede hemorrhagic cystitis; may cause bladder cancer

Azathioprine (Imuran)

May be added if no response to steroid or if on steroid for long period of time. Antagonizes purine metabolism and inhibits synthesis of DNA, RNA, and proteins. May decrease proliferation of immune cells, which results in lower autoimmune activity.

Dosing

Adult

1-2 mg/kg PO qd in single or divided doses

Pediatric

Administer as in adults

Interactions

Toxicity increases with allopurinol, and dose must be reduced; concurrent use with ACE inhibitors may induce severe leukopenia; may increase levels of methotrexate metabolites and decrease effects of anticoagulants, neuromuscular blockers, and cyclosporine

Contraindications

Documented hypersensitivity

Precautions

Pregnancy

D - Fetal risk shown in humans; use only if benefits outweigh risk to fetus

Precautions

Increases risk of neoplasia; caution with liver disease and renal impairment; hematologic toxicities may occur

Methotrexate (Folex PFS, Rheumatrex)

May be added if steroid not effective or if on steroid for prolonged period of time. Unknown mechanism of action in treatment of inflammatory reactions; may affect immune function. Ameliorates symptoms of inflammation (eg, pain, swelling, stiffness). Adjust dose gradually to attain satisfactory response.

Dosing

Adult

7.5-25 mg/wk PO/IM

Pediatric

Not established

Interactions

Oral aminoglycosides may decrease absorption and blood levels of concurrent oral MTX; charcoal lowers levels; coadministration with etretinate may increase hepatotoxicity of MTX; folic acid or its derivatives contained in some vitamins may decrease response to MTX
Coadministration with NSAIDs may be fatal; indomethacin and phenylbutazone can increase MTX plasma levels; may decrease phenytoin serum levels
Probenecid, salicylates, procarbazine, and sulfonamides, including TMP-SMZ, may increase effects and toxicity of MTX; may increase plasma levels of thiopurines

Contraindications

Documented hypersensitivity, alcoholism, hepatic insufficiency, documented immunodeficiency syndromes, preexisting blood dyscrasias (eg, bone marrow hypoplasia, leukopenia, thrombocytopenia, significant anemia)

Precautions

Pregnancy

D - Fetal risk shown in humans; use only if benefits outweigh risk to fetus

Precautions

Monitor CBCs monthly and liver and renal function every 1-3 mo during therapy (monitor more frequently during initial dosing, dose adjustments, or with risk of elevated MTX levels, eg, dehydration); has toxic effects on hematologic, renal, GI, pulmonary, and neurologic systems
Discontinue if significant drop in blood counts; aspirin, NSAIDs, or low-dose steroids may be administered concomitantly with MTX (possibility of increased toxicity with NSAIDs including salicylates has not been tested)

Cyclosporine (Sandimmune, Neoral, Gengraf)

May be added if steroid ineffective or on steroid for prolonged period of time. Cyclic polypeptide that suppresses some humoral immunity and, to a greater extent, cell-mediated immune reactions. For children and adults, base dosing on ideal body weight.

Dosing

Adult

3 mg/kg/d PO qd or divided bid; if creatinine clearance increases by >30%, dosage must be lowered

Pediatric

Administer as in adults

Interactions

Carbamazepine, phenytoin, isoniazid, rifampin, and phenobarbital may decrease concentrations; azithromycin, itraconazole, nicardipine, ketoconazole, fluconazole, erythromycin, verapamil, grapefruit juice, diltiazem, aminoglycosides, acyclovir, amphotericin B, and clarithromycin may increase toxicity; acute renal failure, rhabdomyolysis, myositis, and myalgias increase when taken concurrently with lovastatin

Contraindications

Documented hypersensitivity; uncontrolled hypertension or malignancies; concomitantly with PUVA or UVB radiation in psoriasis (may increase risk of cancer)

Precautions

Pregnancy

C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus

Precautions

May cause hypertension and paresthesias; evaluate renal and liver functions often by measuring BUN, serum creatinine, serum bilirubin, and liver enzymes; may increase risk of infection and lymphoma; reserve IV use only for those who cannot take PO; may cause gout

Infliximab (Remicade)

May be added if steroids and other immunosuppressant drugs are ineffective in achieving or maintaining remission. 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.

Dosing

Adult

3 mg/kg IV (in combination with methotrexate or other immunosuppressants); follow by additional 3 mg/kg IV at 2 and 6 wk after first dose; repeat q8wk thereafter; can be increased to 10 mg/kg IV q4-8wk

Pediatric

Not established

Interactions

None reported

Contraindications

Documented hypersensitivity

Precautions

Pregnancy

C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus

Precautions

May adversely affect normal immune responses and allow development of superinfections (more cases of lymphoma were observed in TNF-a –blockers compared with controls); may increase risk of reactivation of tuberculosis in patients with particular granulomatous infections

Etanercept (Enbrel)

May be added if steroids and other immunosuppressant drugs are ineffective in achieving or maintaining remission. Soluble p75 TNF receptor fusion protein (sTNFR-Ig). Inhibits TNF binding to cell surface receptors, which, in turn, decreases inflammatory and immune responses.

Dosing

Adult

25 mg SC 2 times/wk with or without concomitant administration of MTX
Can be increased to 50 mg SC 2 times/wk

Pediatric

Not established

Interactions

None reported

Contraindications

Documented hypersensitivity; sepsis; concurrent live vaccination

Precautions

Pregnancy

B - Fetal risk not confirmed in studies in humans but has been shown in some studies in animals

Precautions

Serious infections may develop and the therapy should be discontinued if they occur; possible adverse effects include injection site pain, redness and swelling at injection site, and headaches; rare cases of lupuslike symptoms and heart failure have been reported (discontinue treatment if symptoms develop)

Mycophenolate (CellCept, Myfortic)

May be added if steroids and other immunosuppressant drugs are ineffective in achieving or maintaining remission. Inhibits purine synthesis and proliferation of human lymphocytes. Promising published case report of 3 patients with resistant disease treated with mycophenolate mofetil. Reduced toxicity makes this regimen an attractive alternative.

Dosing

Adult

1 g PO bid

Pediatric

Not established

Interactions

In combination with either acyclovir or ganciclovir may result in higher levels for both interacting drugs due to competition for renal tubular excretion; aluminum or magnesium present in some antacids and cholestyramine-containing products may decrease absorption, reducing levels (do not administer together); probenecid may increase levels of mycophenolate; salicylates and azathioprine may increase toxicity; may decrease levonorgestrel AUC; may decrease live virus vaccine immune response; may increase free fraction levels of theophylline when administered in combination with theophylline

Contraindications

Documented hypersensitivity

Precautions

Pregnancy

D - Fetal risk shown in humans; use only if benefits outweigh risk to fetus

Precautions

Increases risk for infection (monitor blood count); severe renal impairment (CrCl <25 mL/min) may have increased adverse effects due to increase free MPA; caution in patients with active peptic ulcer disease; incidence of malignancies and lymphoma is consistent with that reported for other immunosuppressants (0.9%); commonly causes constipation, nausea, diarrhea, urinary tract infection, and nasopharyngitis; rare reports include interstitial lung disorders, colitis, pancreatitis, intestinal perforation, GI hemorrhage, gastric ulcers, duodenal ulcers, and ileus; do not chew, crush, or cut Myfortic tab

Follow-up

Further Outpatient Care

• Follow-up should be with either a primary care physician or 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.

Prognosis

• Takayasu arteritis is associated with substantial morbidity and may be life-threatening.
• Its course usually extends for many years, with varying degrees of activity.
• Approximately 20% of patients have a monophasic and self-limited disease. In others, Takayasu arteritis is progressive or relapsing/remitting and requires immunosuppressive treatment.
• The 15-year survival rate has been reported to be 90-95%.

Miscellaneous

Medicolegal Pitfalls

• Failure to diagnose appropriately
• Failure to document extent of vascular disease

Special Concerns

• Pregnancy may be safer during presumed remission of Takayasu arteritis. It may exacerbate hypertension and/or cardiovascular complications and is a greater risk for both maternal and fetal morbidity and mortality.
• Acknowledgment: I would like to thank my wife, Eisha, and beautiful children Maahum and Nehaal for their love and support in completing this project.

Multimedia

Media file 1: 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.

Media file 2: Characteristic long, tapered narrowing of the distal aorta and iliac vessels. Image courtesy of Robert Cirillo, MD.

Media file 3: 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.

Media file 4: 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.

Media file 5: MRI of thorax of 15-year-old girl with Takayasu arteritis. Note aneurysms of descending aorta. Image courtesy of Christine Hom, MD.

Media file 6: 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.

References

1. Yagi K, Kobayashi J, Yasue S, et al. Four unrelated cases with Takayasu arteritis and CD36 deficiency: possible link between these disorders. J Intern Med. Jun 2004;255(6):688-9. [Medline].

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

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

4. Andrews J, Mason JC. Takayasu's arteritis--recent advances in imaging offer promise. Rheumatology (Oxford). Jan 2007;46(1):6-15. [Medline].

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

6. Joh JH, Kim DK, Park KH, Kim DI. Surgical management of Takayasu's arteritis. J Korean Med Sci. Feb 2006;21(1):20-4. [Medline].

7. Liang P, Hoffman GS. Advances in the medical and surgical treatment of Takayasu arteritis. Curr Opin Rheumatol. Jan 2005;17(1):16-24. [Medline].

8. Lie JT. Pathology of isolated nonclassical and catastrophic manifestations of Takayasu arteritis. Int J Cardiol. Oct 1 1998;66 Suppl 1:S11-21. [Medline].

9. Numano F. Takayasu arteritis, Buerger disease and inflammatory abdominal aortic aneurysms: is there a common pathway in their pathogenesis?. Int J Cardiol. Oct 1 1998;66 Suppl 1:S5-10. [Medline].

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

11. Rizzi R, Bruno S, Stellacci C, Dammacco R. Takayasu's arteritis: a cell-mediated large-vessel vasculitis. Int J Clin Lab Res. 1999;29(1):8-13. [Medline].

12. Schmidt WA, Blockmans D. Use of ultrasonography and positron emission tomography in the diagnosis and assessment of large-vessel vasculitis. Curr Opin Rheumatol. Jan 2005;17(1):9-15. [Medline].

13. Seo P, Stone JH. Large-vessel vasculitis. Arthritis Rheum. Feb 15 2004;51(1):128-39. [Medline].

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

15. Tanaka F, Kawakami A, Iwanaga N, Tamai M, Izumi Y, Aratake K. Infliximab is effective for Takayasu arteritis refractory to glucocorticoid and methotrexate. Intern Med. 2006;45(5):313-6. [Medline].

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

Keywords

Takayasu arteritis, TA, pulseless disease, Takayasu's disease, Takayasu's arteritis, Takayasu disease, aortic arch syndrome, middle aortic syndrome stenotic lesions, angioplasty, renovascular stenosis, coronary artery stenosis, extremity claudication, cerebral ischemia, critical stenosis, aortic regurgitation, thoracic aneurysm, abdominal aneurysm, percutaneous transluminal coronary angioplasty, PTCA

Contributor Information and Disclosures

Author

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, and American Federation for Medical Research
Disclosure: Nothing to disclose.

Coauthor(s)

Robert E Wolf, MD, PhD, Professor Emeritus, Department of Medicine, Louisiana State University Health Sciences Center at Shreveport; Chief, Rheumatology Section, Medical Service, Overton Brooks Veterans Administration Medical Center of Shreveport
Robert E Wolf, MD, PhD is a member of the following medical societies: American College of Rheumatology, Arthritis Foundation, and Society for Leukocyte Biology
Disclosure: Nothing to disclose.

Pharmacy Editor

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

Managing Editor

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.

CME Editor

Alex J Mechaber, MD, FACP, Assistant Dean for Medical Curriculum, Associate Professor of Medicine, Division of General Internal Medicine, University of Miami Miller School of Medicine
Alex J Mechaber, MD, FACP is a member of the following medical societies: Alpha Omega Alpha, American College of Physicians-American Society of Internal Medicine, and Society of General Internal Medicine
Disclosure: Nothing to disclose.

Chief Editor

Herbert S Diamond, MD, Professor of Medicine, Temple University School of Medicine; 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, and Phi Beta Kappa
Disclosure: medifocus Honoraria Review panel membership; health dialogs Honoraria Consulting; Merck, Amgen, Biogen, Zimmer, Wyeth, Johnson&Johnson, Stryker, Medtronic, Zimmer.Abbott,  Ownership interest Other; West Penn Allegheny Health System Consulting fee Consulting; Alpharma Honoraria Consulting; Proctor&Gamble Grant/research funds Independent contractor

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