Aortitis: Symptoms, Types, Causes and Treatment

Aortitis refers to inflammation of the aorta, the main artery carrying blood from the heart to the rest of the body. This condition can be subtle and hard to detect, but it has potentially life-threatening consequences, including aneurysm and dissection. Understanding the symptoms, types, causes, and treatment strategies is crucial for early diagnosis and effective management. In this article, we break down the complexities of aortitis into clear, actionable insights based on the latest research.

Symptoms of Aortitis

Aortitis is notorious for its vague and non-specific symptoms. Many patients experience only mild discomfort or generalized symptoms, which often leads to delays in diagnosis. Recognizing the key warning signs can help clinicians and patients seek timely intervention.

Symptom	Description	Frequency/Context	Source(s)
Pain	Dorsal, lumbar, abdominal, or chest pain	Common at diagnosis, sometimes diffuse	1, 2, 3, 6, 9, 11
Constitutional	Fever, weight loss, fatigue	Often present, but variable	2, 3, 6, 11
Vascular Signs	Aortic insufficiency, vascular insufficiency	May indicate progression or complications	1, 6, 11
PMR Features	Polymyalgia rheumatica (muscle pain, stiffness)	Frequent, especially with GCA	2
Elevated Markers	Raised erythrocyte sedimentation rate (ESR), C-reactive protein (CRP)	Seen in most cases	2, 3, 6, 11

Table 1: Key Symptoms

Common Presentations

Aortitis can manifest with a variety of symptoms, making it a diagnostic challenge. Pain is the most frequent complaint, and it can be localized (chest, back, abdomen) or more diffuse, such as lower limb or low back pain. Some patients experience symptoms that mimic other conditions, like atypical polymyalgia rheumatica (PMR), especially in older adults with giant cell arteritis (GCA) 1, 2.

Constitutional and Systemic Signs

Fever, weight loss, and general malaise are common but non-specific. These constitutional symptoms often occur alongside elevated markers of inflammation in blood tests, such as ESR and CRP 2, 3, 6, 11. In some cases, vascular signs like aortic insufficiency or features of vascular insufficiency (for example, weak pulses or limb ischemia) may reveal the diagnosis or indicate progression.

Red Flags for Clinicians

• Atypical PMR features (muscle pain and stiffness not explained by classic PMR)
• Unexplained low back or limb pain
• Constitutional symptoms with elevated inflammatory markers
• Signs of vascular compromise or new aortic insufficiency

These should prompt consideration of aortitis, especially when standard diagnostics fail to explain symptoms 2.

Types of Aortitis

Aortitis is not a single disease, but a syndrome caused by various underlying conditions. Classification is based on the underlying etiology, histologic patterns, and clinical associations.

Type	Key Characteristics	Common Age Group / Demographics	Source(s)
Giant Cell Arteritis	Granulomatous, often with PMR, older adults	>50 years, mainly women	1, 4, 5, 7, 8
Takayasu Arteritis	Granulomatous, affects large arteries	Young women (<40 years)	4, 7, 8
Isolated Aortitis	No systemic disease, histologically similar to GCA	Older adults, often women	7, 8
Infectious Aortitis	Bacterial, fungal, or viral origin	All ages, often with risk factors	9, 11, 16
IgG4-related Aortitis	Lymphoplasmacytic infiltrate, systemic or localized	Middle-aged/older adults	10, 13

Table 2: Main Types of Aortitis

Giant Cell Arteritis (GCA) Aortitis

GCA is the most common type of non-infectious aortitis, particularly in people over 50. It frequently coexists with PMR and predominantly affects women. GCA-associated aortitis increases the risk of aneurysm and dissection, especially if symptoms are present at diagnosis 1, 5, 7, 8.

Takayasu Arteritis

This type primarily affects young women under 40, often in Asian populations. The inflammation involves the aorta and its major branches, leading to stenosis or aneurysm formation. Takayasu arteritis is rare in older adults 4, 7, 8.

Isolated Aortitis

Isolated aortitis refers to inflammation confined to the aorta without evidence of systemic disease. It is often discovered incidentally during surgery or imaging for aneurysms. Most affected patients are older women, and the clinical course is generally benign compared to GCA or Takayasu 7, 8.

Infectious Aortitis

Less common but potentially devastating, infectious aortitis can be caused by bacteria (such as Salmonella, Staphylococcus, Clostridium), fungi, or viruses. It often develops in individuals with predisposing conditions like diabetes, immunosuppression, or atherosclerosis. Infectious aortitis typically presents acutely and may rapidly progress to aneurysm and rupture if not treated promptly 9, 11, 16.

IgG4-Related Aortitis

A newer entity, IgG4-related aortitis is characterized by a lymphoplasmacytic infiltrate and is often part of a systemic IgG4-related disease. It can mimic other forms of aortitis and is important to recognize because of its unique treatment implications 10, 13.

Causes of Aortitis

The root causes of aortitis are diverse, spanning both infectious and non-infectious origins. Understanding these helps guide treatment and prognosis.

Cause Category	Examples/Details	Notes or Risk Factors	Source(s)
Autoimmune/Inflammatory	GCA, Takayasu, Rheumatoid arthritis, SLE, Behçet's, Sarcoidosis, IgG4	Most common causes, often systemic	3, 4, 5, 7, 10, 11, 13
Infectious	Bacteria (Salmonella, Staph, Clostridium, Syphilis), Fungi, Viruses	Risk: immunosuppression, atherosclerosis	9, 11, 16
Idiopathic	No clear underlying cause	Often discovered incidentally	7, 8, 11
Drug-Induced	Granulocyte colony-stimulating factor (G-CSF), others	Rare, often in chemotherapy patients	14

Table 3: Principal Causes of Aortitis

Autoimmune and Inflammatory Causes

The majority of aortitis cases are related to autoimmune or inflammatory diseases:

• Giant Cell Arteritis (GCA): Most frequent in older adults, often with PMR features.
• Takayasu Arteritis: Younger women, often with systemic features.
• Other autoimmune diseases: Rheumatoid arthritis, systemic lupus erythematosus (SLE), Behçet's disease, sarcoidosis, and IgG4-related disease all have recognized associations with aortitis 3, 4, 5, 7, 10, 11, 13.

Infectious Causes

Infectious aortitis is less common but highly dangerous. Bacterial pathogens (such as Salmonella, Clostridium, and Staphylococcus) are the most frequent, often infecting the aorta via the bloodstream or direct extension from nearby infections. Syphilitic aortitis, once common, is now rare. Fungal and viral causes are less frequent but important in immunocompromised patients 9, 11, 16.

Idiopathic and Drug-Induced Aortitis

Some cases have no identifiable cause (idiopathic). Increasingly, drug-induced aortitis is being recognized, particularly with granulocyte colony-stimulating factor (G-CSF) therapy in cancer patients. The mechanism is not entirely understood, but studies show a marked increase in risk with G-CSF exposure 14.

Treatment of Aortitis

Managing aortitis requires a tailored approach, depending on the underlying cause, disease activity, and presence of complications. Both medical and surgical options may be needed.

Treatment	Indication/Details	Outcomes/Notes	Source(s)
Corticosteroids	First-line for non-infectious aortitis	Effective in most; relapses possible	7, 15, 17
Immunosuppressants	For refractory/relapsing disease	Methotrexate, azathioprine, biologics (e.g., tocilizumab)	15, 17
Antibiotics	Essential for infectious aortitis	Long-term, often combined with surgery	9, 16
Surgery	Aneurysm, dissection, severe disease	Mortality risk; needed for complications	5, 8, 16, 17
Endovascular Repair	Alternative to open surgery in select cases	Lower morbidity, but risk of infection	16, 17

Table 4: Main Treatment Strategies

Medical Management

Corticosteroids

High-dose corticosteroids are the first-line therapy for non-infectious aortitis (such as GCA and Takayasu). They reduce inflammation and control symptoms in most patients. However, relapses are common, especially if steroids are tapered too quickly 7, 15, 17.

Immunosuppressants and Biologic Therapy

For patients with refractory or relapsing disease, additional immunosuppressive agents such as methotrexate or azathioprine may be added. Biologic therapies targeting interleukin-6 (such as tocilizumab) have shown efficacy in steroid-resistant cases and can help reduce steroid dependence 15.

Treatment of Infectious Aortitis

Infectious aortitis is a medical emergency. Prompt treatment with intravenous bactericidal antibiotics is mandatory, tailored to the specific pathogen. Surgery is often required to repair or replace the affected aorta, especially if there is an aneurysm, dissection, or rupture 9, 16.

• Open Surgical Repair: Preferred for potentially curative treatment, especially in cases of bacterial aortitis (e.g., Clostridium septicum).
• Endovascular Repair: Considered in patients unfit for open surgery but with higher risk for graft infection and lower long-term survival 16.

Surgical and Endovascular Interventions

Surgical intervention is indicated for:

• Symptomatic aneurysm or dissection
• Rapidly expanding aortic diameter
• Vascular compromise

Endovascular procedures are increasingly favored due to lower morbidity, but may not be suitable for all patients, especially in the setting of active infection 5, 8, 16, 17.

Monitoring and Follow-Up

• Imaging: Regular surveillance with CT, MRI, or PET-CT to monitor disease activity, detect aneurysm formation, and assess response to treatment 3, 6, 11.
• Laboratory: Inflammatory markers (ESR, CRP) help monitor activity but should not be used in isolation.

Conclusion

Aortitis is a complex, multifaceted disease that requires a high index of suspicion for timely diagnosis and effective management. Key takeaways include:

• Symptoms are often vague and non-specific, with pain, constitutional symptoms, and elevated inflammatory markers as common features.
• Types of aortitis are diverse, with non-infectious (GCA, Takayasu, isolated, IgG4-related) and infectious forms, each with unique epidemiology and implications.
• Causes range from autoimmune and infectious to idiopathic and drug-induced, necessitating a careful clinical and laboratory work-up.
• Treatment is determined by etiology and disease severity, with corticosteroids and immunosuppressants for non-infectious forms, and antibiotics plus surgery for infectious cases.
• Close monitoring and individualized care are essential for optimal outcomes.

By recognizing the warning signs and understanding the underlying causes and treatment strategies, clinicians can improve outcomes for patients with this serious but often overlooked disease.