What is the appropriate treatment for aortitis, including both infectious and non‑infectious causes?

Treatment of Aortitis

The treatment of aortitis depends critically on distinguishing infectious from non-infectious causes: infectious aortitis requires immediate antimicrobial therapy targeting the most common pathogens (Staphylococcus aureus and Salmonella species), while non-infectious aortitis demands high-dose glucocorticoids (40–60 mg prednisone daily or 1 mg/kg up to 80 mg) initiated immediately without awaiting biopsy confirmation, combined with steroid-sparing immunosuppression to prevent irreversible vascular damage. 1

Initial Diagnostic Imperative

Before initiating treatment, you must differentiate infectious from inflammatory aortitis, as the therapeutic approaches are fundamentally opposed:

• Obtain cross-sectional imaging immediately (CT angiography, MR angiography, or FDG-PET) to confirm aortic wall thickening, enhancement, edema, stenosis, or aneurysm formation 1, 2
• MR angiography with gadolinium is preferred for detecting active inflammation, demonstrating late gadolinium enhancement, T2-weighted wall edema, and wall thickening 1
• Blood cultures and serologic testing are mandatory to exclude infectious causes, particularly Staphylococcus aureus, Salmonella, Treponema pallidum (syphilis), Mycobacterium tuberculosis, and fungal pathogens 3, 1

Treatment of Infectious Aortitis

Infectious aortitis is a surgical emergency with high mortality if untreated:

• Initiate broad-spectrum intravenous antibiotics immediately upon suspicion, then narrow therapy based on culture results and sensitivities 4, 5
• The most common pathogens are Staphylococcus aureus and Salmonella species, followed by Streptococcus pneumoniae and Escherichia coli 3
• For syphilitic aortitis, administer IV penicillin G after serologic confirmation with both nontreponemal (RPR/VDRL) and treponemal tests 6
• Fungal infections (Candida, Aspergillus) occur in immunocompromised patients and require antifungal therapy 3
• Surgical intervention is often necessary to debride infected tissue, repair or replace the affected aortic segment, and drain abscesses, particularly when aneurysm formation or rupture is imminent 3, 4

Critical Pitfall

Do not delay antimicrobial therapy while awaiting definitive microbiologic diagnosis—empiric broad-spectrum coverage must begin immediately given the high mortality of untreated infectious aortitis. 5

Treatment of Non-Infectious (Inflammatory) Aortitis

Immediate Glucocorticoid Therapy

Start treatment immediately upon clinical suspicion—do not wait for biopsy or imaging confirmation:

• High-dose oral prednisone 40–60 mg daily (or 1 mg/kg up to 80 mg) is the initial therapy for all non-infectious aortitis 1
• For life- or organ-threatening manifestations (vision loss, stroke, myocardial ischemia, limb ischemia), administer IV methylprednisolone 500–1000 mg daily for 3–5 days before transitioning to oral therapy 1
• Glucocorticoid monotherapy should be avoided except in mild disease or diagnostic uncertainty, as combination therapy with steroid-sparing agents markedly reduces long-term toxicity and improves outcomes 1, 2

Steroid-Sparing Immunosuppression

Initiate a non-glucocorticoid immunosuppressive agent early to reduce cumulative steroid exposure:

For Giant Cell Arteritis (GCA)

• Tocilizumab (IL-6 receptor antagonist) is preferred for refractory/relapsing disease or when glucocorticoid-related adverse-event risk is high, as it lowers cumulative glucocorticoid exposure and relapse rates 1
• Methotrexate is an acceptable alternative when tocilizumab is unavailable or contraindicated 1

For Takayasu Arteritis

• Initiate a steroid-sparing agent at diagnosis: methotrexate 20–25 mg weekly (especially in children) or azathioprine 2 mg/kg/day 1
• TNF inhibitors (infliximab or adalimumab) may be used when rapid disease control is required or conventional agents are contraindicated 1
• For refractory disease after glucocorticoids plus conventional agents, add a TNF inhibitor before considering tocilizumab, based on observational data showing higher remission rates with TNF blockade 1

For Pediatric Patients

• Methotrexate is the preferred first-line steroid-sparing agent in children due to superior tolerability 1
• For extensive disease crossing the diaphragm, consider cyclophosphamide induction followed by methotrexate maintenance 1

Adjunctive Antiplatelet Therapy

• The European League Against Rheumatism no longer recommends routine antiplatelet or anticoagulant therapy for large-vessel vasculitis unless there is a separate indication 1
• Low-dose aspirin (75–150 mg daily) may be considered in patients with active disease involving critical cranial, vertebrobasilar, or flow-limiting carotid/vertebral arteries to reduce ischemic events, with caution regarding bleeding risk 1

Glucocorticoid Tapering Strategy

• After achieving remission for 6–12 months, taper glucocorticoids completely rather than maintaining a low-dose regimen 1
• Continue the steroid-sparing immunosuppressive agent throughout and after the taper 1

Disease Activity Monitoring

Lifelong clinical surveillance is mandatory because vascular remodeling can progress even when patients appear clinically quiescent:

• At each visit, obtain four-extremity blood pressures, perform a vascular examination for new bruits or pulse deficits, and assess constitutional symptoms (fever, weight loss, fatigue) and vascular symptoms (claudication, hypertension) 1
• Inflammatory markers (ESR, CRP) are elevated in only ~50% of active disease episodes and must not be used in isolation—combine clinical assessment with imaging 1, 7
• Imaging surveillance (MRI/CT angiography or FDG-PET) every 3–6 months during active or early disease (longer intervals once quiescent) to detect wall edema, contrast enhancement, increased thickness, or supraphysiologic FDG uptake 1, 2
• New arterial stenosis or wall thickening in previously unaffected territories mandates escalation of immunosuppression, even if asymptomatic 1

Critical Pitfall

Do not rely solely on inflammatory markers for disease activity assessment—they are normal in half of active cases. Always combine clinical evaluation with imaging. 1, 7

Surgical and Interventional Management

Timing of surgical intervention is critical and depends on disease activity:

• Elective revascularization (bypass, angioplasty, stenting) should be postponed until disease is quiescent, as operating during active inflammation is linked to significantly poorer outcomes 1, 2
• If surgery is unavoidable for life- or organ-threatening ischemia, administer high-dose glucocorticoids peri-procedurally and ensure joint decision-making between vascular surgeons and rheumatologists 1
• For renovascular hypertension in Takayasu arteritis, first manage medically with antihypertensives and immunosuppression; reserve renal artery revascularization for refractory hypertension or progressive renal dysfunction despite optimized therapy 1
• For syphilitic aortitis with ascending aortic aneurysm, surgical intervention with aortic valve replacement (if indicated) is required, with timing allowing for planned surgical repair after antibiotic initiation 6

Critical Pitfall

Do not perform elective surgery during active inflammation—outcomes are markedly worse. Achieve clinical remission first. 1, 2

Special Considerations for Ankylosing Spondylitis

• Aortic root and aortic valve involvement occur in up to 80% of patients with ankylosing spondylitis, with aortic valvular regurgitation present in nearly half 3
• Treatment of aortic root expansion and aortic valvular abnormalities is the same as for other conditions (i.e., surgical repair when indicated by standard criteria) 3

Lifestyle Modifications

• Smoking cessation is strongly recommended due to its association with increased mortality in aortitis patients 2
• Regular cardiovascular exercise is beneficial, but contact/competitive sports and isometric exercise should be avoided 2
• Patients should wear a medical alert bracelet indicating predisposition to aortic emergencies and seek immediate medical care for any unexpected chest discomfort 2

Common Pitfalls to Avoid

• Do not delay glucocorticoid initiation while awaiting biopsy or imaging in suspected non-infectious aortitis—early treatment prevents irreversible vascular damage 1
• Do not use glucocorticoid monotherapy except for mild disease—combination therapy reduces toxicity and improves outcomes 1
• Do not miss infectious aortitis—it requires prompt antimicrobial therapy and carries high mortality if untreated 1, 5
• Do not discontinue monitoring in clinical remission—silent vascular remodeling can occur, particularly in isolated aortitis where dissection risk is 16.6% compared to 1.96% in other types 7
• Do not rely solely on inflammatory markers—they are normal in half of active cases 1, 7