What is the recommended management for aortitis?

Management of Aortitis

Immediately initiate high-dose oral glucocorticoids (40–60 mg/day prednisone-equivalent) combined with a non-glucocorticoid immunosuppressive agent as soon as aortitis is suspected, even before diagnostic confirmation, to prevent irreversible vascular complications including blindness, stroke, aneurysm formation, and vascular stenosis. 1, 2

Diagnostic Confirmation and Etiology Assessment

Before finalizing treatment, confirm the diagnosis with imaging (CT angiography, MR angiography, or FDG-PET) demonstrating aortic wall thickening, enhancement, edema, or stenosis/aneurysm formation 1. The imaging modality choice depends on clinical context:

• MR angiography with gadolinium is preferred for detecting active inflammation, showing late gadolinium enhancement, vessel wall edema on T2-weighted imaging, and wall thickening 1
• FDG-PET identifies metabolically active inflammation with supraphysiologic FDG uptake and is superior for monitoring treatment response 1, 2
• CT angiography provides comprehensive anatomic assessment of the entire aorta and branch vessels but characterizes active inflammation poorly 1

Distinguish infectious from non-infectious aortitis immediately, as management differs fundamentally 1:

Infectious Aortitis (Medical Emergency)

• Most commonly caused by Staphylococcus aureus and Salmonella species 1
• Other pathogens include Treponema pallidum (syphilis), Mycobacterium tuberculosis, Candida, Aspergillus, and gram-negative organisms 1
• Obtain blood cultures, consider tissue biopsy if feasible, and initiate broad-spectrum antimicrobial therapy immediately 1, 3
• Surgical intervention is often necessary due to rapid progression and high mortality 1, 3

Non-Infectious Aortitis

Determine the specific vasculitis subtype based on age and vascular distribution 1:

Age >60 years: Giant cell arteritis (GCA) is most common 1

• Look for temporal headache, jaw claudication (positive likelihood ratio 4.90), limb claudication (positive likelihood ratio 6.01), temporal artery abnormalities, polymyalgia rheumatica symptoms 4
• ESR >100 mm/h (positive likelihood ratio 3.11) and thrombocytosis >400×10³/μL (positive likelihood ratio 3.75) support GCA 4
• Obtain temporal artery biopsy >1 cm within 2 weeks of starting glucocorticoids to maximize diagnostic yield 4

Age <60 years: Takayasu arteritis is most common 1

• Assess for diminished/absent pulses, blood pressure discrepancy >10 mmHg between arms, vascular bruits over subclavian arteries or aorta 2
• Constitutional symptoms (fever, weight loss, fatigue, night sweats) often precede vascular manifestations 2
• Obtain four-extremity blood pressures at every assessment 2

Other causes include granulomatosis with polyangiitis, systemic lupus erythematosus, rheumatoid arthritis, Behçet disease, sarcoidosis, IgG4-related disease, and idiopathic/clinically isolated aortitis 1

Initial Medical Management for Non-Infectious Aortitis

Glucocorticoid Therapy

Start high-dose oral prednisone 40–60 mg daily (or 1 mg/kg/day, maximum 80 mg) immediately when aortitis is suspected, without waiting for biopsy or imaging confirmation 1, 2, 4. This applies to both GCA and Takayasu arteritis.

For life- or organ-threatening manifestations (vision loss, stroke, myocardial ischemia, limb ischemia), administer IV methylprednisolone 500–1,000 mg/day for 3–5 days before transitioning to high-dose oral therapy 2, 5. However, IV pulse therapy is not superior to high-dose oral glucocorticoids for non-emergent presentations 2.

Steroid-Sparing Immunosuppression

Do not use glucocorticoid monotherapy except in mild disease or diagnostic uncertainty—combination therapy significantly reduces long-term glucocorticoid toxicity and improves outcomes 2, 5.

For Takayasu arteritis: Initiate a non-glucocorticoid immunosuppressive agent simultaneously with glucocorticoids at diagnosis 1, 2:

• Methotrexate 20–25 mg weekly is the preferred first-line agent, especially in children due to superior tolerability 2
• Azathioprine 2 mg/kg/day is an acceptable alternative 2
• TNF inhibitors (infliximab or adalimumab) may be used as initial adjunctive therapy when rapid disease control is needed or conventional agents are contraindicated 2

For GCA: Adjunctive therapy is recommended in selected patients 1:

• Refractory or relapsing disease
• Presence of increased risk for glucocorticoid-related adverse events
• Tocilizumab (IL-6 receptor antibody) is preferred to reduce cumulative glucocorticoid dose and relapse frequency 1, 4
• Methotrexate may be used as an alternative 1

Adjunctive Antiplatelet Therapy

Add low-dose aspirin (75–150 mg daily) for patients with active disease and critical cranial, vertebrobasilar, or flow-limiting carotid/vertebral artery involvement 2, 4. This reduces ischemic event risk but increases bleeding risk, so use cautiously after surgical procedures 2.

EULAR no longer recommends routine antiplatelet or anticoagulant therapy for large vessel vasculitis unless indicated for other reasons 1.

Management of Refractory Disease

For patients failing glucocorticoids plus conventional immunosuppressants (methotrexate or azathioprine), add a TNF inhibitor rather than tocilizumab as the next therapeutic step 2. This recommendation is based on broader clinical experience and observational studies showing higher remission rates and fewer relapses with TNF blockade 2.

Reserve tocilizumab for cases where TNF inhibitors are contraindicated, ineffective, or not tolerated 2. A single randomized trial in Takayasu arteritis failed to meet its primary efficacy endpoint, although subsequent observational studies suggest benefit in selected refractory patients 2.

Glucocorticoid Tapering Strategy

After achieving remission for 6–12 months, taper glucocorticoids completely rather than maintaining long-term low-dose therapy 1, 2, 5. Continue the non-glucocorticoid immunosuppressive agent during and after the glucocorticoid taper 2, 5.

Disease Activity Monitoring

Lifelong clinical surveillance is mandatory because vascular remodeling can progress even when patients appear clinically quiescent 2, 5.

At every visit:

• Obtain four-extremity blood pressures to detect discrepancies 2, 5
• Perform vascular examination for new bruits or pulse deficits 2, 5
• Assess constitutional symptoms (fever, weight loss, fatigue) and vascular symptoms (claudication, hypertension) 2
• Measure inflammatory markers (ESR, CRP) alongside clinical assessment 1, 2

Critical Pitfall: Inflammatory Markers Are Unreliable

Inflammatory markers are elevated in only ~50% of active disease episodes and must never be used in isolation to assess activity 2, 5. Clinical assessment combined with imaging is essential 2. Isolated elevation of inflammatory markers without clinical symptoms does not warrant escalation of immunosuppressive therapy—instead, observe and monitor more frequently 4.

Imaging Surveillance

Perform noninvasive imaging (MRI/CT angiography or FDG-PET) every 3–6 months during active or early disease, with longer intervals once disease is established as quiescent 2, 5. Findings indicating active inflammation include:

• Vascular wall edema, contrast enhancement, increased wall thickness on MR/CT 2
• Supraphysiologic FDG uptake on PET 2

New arterial stenosis or vessel wall thickening in new territories on imaging warrants immunosuppressive therapy escalation, even if clinically asymptomatic 2.

Surgical and Interventional Management

Delay elective revascularization procedures (bypass grafting, angioplasty, stenting) until disease is quiescent whenever possible—operating during active inflammation is associated with significantly worse outcomes 1, 2, 5. Observational studies consistently demonstrate this principle 2.

If surgery is unavoidable due to life- or organ-threatening ischemia:

• Administer high-dose glucocorticoids in the peri-procedural period 2, 5
• Ensure collaborative decision-making between vascular surgeons and rheumatologists for all surgical interventions 2, 5

Management of Renovascular Hypertension in Takayasu Arteritis

For renal artery stenosis causing renovascular hypertension, initial management is medical therapy with antihypertensive drugs plus immunosuppressive therapy 2, 5. Control vascular inflammation first to improve renal blood flow and prevent progression 5.

Reserve renal artery revascularization (angioplasty/stenting or surgical bypass) for hypertension refractory to optimized medical management AND optimized immunosuppressive therapy, or progressive worsening of renal function despite medical therapy 2, 5.

Special Populations: Pediatric Patients

Methotrexate is the preferred first-line steroid-sparing agent in children due to better tolerability 2. Consider cyclophosphamide induction followed by methotrexate maintenance for children with widespread disease involving both sides of the diaphragm 2. Alternate steroid dosing regimens (IV pulse with low daily oral dosing) may improve compliance and reduce growth impairment 2.

Common Pitfalls to Avoid

• Do not delay glucocorticoid initiation waiting for biopsy or imaging confirmation 1, 2, 4
• Do not rely on inflammatory markers alone for disease activity assessment—they are normal in 50% of active cases 2, 5
• Do not use glucocorticoid monotherapy except for mild disease or uncertain diagnosis—combination therapy reduces glucocorticoid toxicity 2, 5
• Do not perform elective surgery during active inflammation—outcomes are significantly worse 2, 5
• Do not discontinue monitoring in clinical remission—vascular changes occur when disease appears quiescent 2, 5
• Do not miss infectious aortitis—it requires antimicrobial therapy, not immunosuppression, and has high mortality if untreated 1, 3