Excerpt for Inflamed Aorta, (Aortitis) A Simple Guide To The Condition, Diagnosis, Treatment And Related Conditions by , available in its entirety at Smashwords

Inflamed Aorta,






The Condition,




Related Conditions


Dr Kenneth Kee

M.B.,B.S. (Singapore)

Ph.D (Healthcare Administration)

Copyright Kenneth Kee 2018 Smashwords Edition

Published by Kenneth Kee at


This book is dedicated

To my wife Dorothy

And my children

Carolyn, Grace

And Kelvin

This book describes Aortitis, Diagnosis and Treatment and Related Diseases which is seen in some of my patients in my Family Clinic.

(What The patient Need to Treat Aortitis)

This eBook is licensed for your personal enjoyment only. This eBook may not be re-sold or given away to other people. If you would like to share this book with another person, please purchase an additional copy for each reader.

If you’re reading this book and did not purchase it, or it was not purchased for your use only, then please return to and purchase your own copy.

Thank you for respecting the hard work of this author.


I have been writing medical articles for my blog (A Simple Guide to Medical Disorder) for the benefit of my patients since 2007.

My purpose in writing these simple guides was for the health education of my patients.

Health Education was also my dissertation for my Ph.D (Healthcare Administration).

I then wrote an autobiolographical account of his journey as a medical student to family doctor on his other blog

This autobiolographical account “A Family Doctor’s Tale” was combined with my early “A Simple Guide to Medical Disorders” into a new Wordpress Blog “A Family Doctor’s Tale” on

From which many free articles from the blog was taken and put together into 800 eBooks.

Some people have complained that the simple guides are too simple.

For their information they are made simple in order to educate the patients.

The later books go into more details of medical disorders.

The first chapter is always from my earlier blogs which unfortunately tends to have typos and spelling mistakes.

Since 2013, I have tried to improve my spelling and writing.

As I tried to bring the patient the latest information about a disorder or illness by reading the latest journals both online and offline, I find that I am learning more and improving on my own medical knowledge in diagnosis and treatment for my patients.

Just by writing all these simple guides I find that I have learned a lot from your reviews (good or bad), criticism and advice.

I am sorry for the repetitions in these simple guides as the second chapters onwards have new information as compared to my first chapter taken from my blog.

I also find repetition definitely help me and maybe some readers to remember the facts in the books more easily.

I apologize if these repetitions are irritating to some readers.

Chapter 1

Inflamed Aorta (Aortitis)

What is Inflamed Aorta (Aortitis)?

Aortitis is the all-encompassing term given to inflammation of the wall of the aorta.

The most frequent causes of aortitis are:

1. The large vessel vasculitides,

2. Giant cell arteritis (GCA) and

3. Takayasu arteritis

Infectious aortitis is a rare but possibly life-threatening disorder.

Aortitis is also linked with several other rheumatologic diseases.

In some cases, aortitis is an incidental finding at the time of histopathology examination after the surgery for aortic aneurysm.

As the medical manifestation of aortitis is highly different, the cardiovascular doctor must have a high index of suspicion to form an accurate diagnosis of this disorder in a timely manner

It is documented that there are only 1–3 new cases of aortitis per year per million people in the United States and Europe.

Aortitis is most frequent in people 10 to 40 years of age.

What are the causes of Aortitis?


This inflammation has a number of possible causes, such as trauma, viral or bacterial infections (notably, syphilis), and certain immune disorders and connective tissue diseases.

Aortitis is most often seen in patients with autoimmune vasculitis (giant cell arteritis, Takayasu's arteritis), polymyalgia rheumatica, and rheumatoid arthritis.

IgG4-related disease has more recently been identified as a cause of aortitis, and also as a cause of peri-aortitis (inflammation surrounding the aorta).


Large-Vessel Vasculitis

1. Giant cell arteritis (temporal arteritis)

2. Takayasu arteritis

3. Rheumatoid arthritis

4. Systemic lupus erythematosis

HLA-B27 associated spondyloarthropathies

1. Ankylosing spondylitis

2. Reiter syndrome

Other vasculitides

1. ANCA-associated vasculitides

a. Wegener's arteritis

b. Polyarteritis nodosum

c. Microscopic polyangiitis

2. Behcet's disease

3. Cogan syndrome

4. Relapsing polychondritis

What are the symptoms of Aortitis?


The medical manifestation of aortitis ranges across a spectrum of symptoms and medical signs, ranging from back or abdominal pain with fever to acute severe aortic insufficiency to an incidentally-identified large thoracic aortic aneurysm.

Acute aortic syndromes (aortic dissection and rupture) can occur in persons with aortitis.

Inflammation-linked thrombus formation in the aortic lumen with peripheral embolization has also been documented.

The underlying cause of the aortitis and its concomitant presentations determine the presenting symptoms.

The site of aortic inflammation (e.g., ascending thoracic versus abdominal aorta) and the occurrence of coexisting arteritis in other blood vessels also determine medical manifestation.

Because of the varied manifestation of aortitis, and the often non-specific nature of its symptoms and signs, the index of suspicion of the examining doctor must be high to form an accurate diagnosis in a timely manner

1. Back or abdominal pain with fever

2. Systemic inflammatory syndrome due to vasculitis

3. Aneurysmal disease

a. Thoracic aortic aneurysm

b. Abdominal aortic aneurysm

4. Cardiac abnormalities

a. Aortic insufficiency (acute or chronic)

b. Stable angina pectoris or acute coronary syndrome due to coronary involvement

5. Aortic thrombosis with distal embolization

6. Aortic dissection or rupture (rare)

7. Upper and lower extremity claudication with pulse deficits

8. Hypertension in a young patient (Takayasu arteritis)

There is a wide range of symptoms that are dependent on the location of the aortic inflammation or associated disorder.

A few symptoms documented in patients are:

1. Fever,

2. Chills,

3. Muscle pains, and

4. Malaise (general discomfort).

Also, hypertension (high blood pressure) may happen.

Hypertension occurs when the renal artery becomes narrow and the elasticity of the aorta and branches is decreased.

How is Aortitis Diagnosed?


While the diagnosis of aortitis is suspected on the basis of medical manifestation, expedient imaging of the entire aorta with a proper method is important to establish the diagnosis.

Modern imaging tools for the aorta are:

1. Computed tomography angiography (CT),

2. Magnetic resonance angiography (MRA), and

3. Ultrasonography.

4. Positron emission tomography (PET) scanning has emerged for targeted imaging of vascular inflammation, and may be especially useful when combined with traditional cross sectional imaging modalities.

The initial evaluation of a patient with suspected aortitis should be:

1. Markers of inflammation, e.g., erythrocyte sedimentation rate (ESR) and c-reactive protein (CRP),

2. A full blood count,

3. Assessment of kidney and liver function, and

4. Blood cultures to exclude the unlikely but critical diagnosis of infectious aortitis.

Additional laboratory testing should be based upon the medical evaluation of the patient and the differential diagnosis of the underlying cause.

A rheumatologic panel may be helpful in the proper medical setting such as:

1. Anti-nuclear antibodies,

2. Anti-neutrophil cytoplasmic antibodies, and

3. Rheumatoid factor.

Skin testing for tuberculosis and serologic testing for syphilis should be reserved for those rare cases in which the clinical suspicion of these very unlikely disorders is high.

In cases of aortitis linked with retroperitoneal fibrosis, secondary causes such as lymphoma should be suspected, and a biopsy should be taken if there is medical suspicion of malignancy on the basis of medical manifestation and imaging studies.

While ESR and CRP are typically markedly elevated in cases of aortitis due to GCA and other systemic vasculitides, these inflammatory markers may be unreliable for prediction of disease activity among patients with Takayasu arteritis.

Accordingly, the American College of Rheumatology diagnostic criteria for GCA is elevated ESR, while the criteria for Takayasu arteritis do not indicate elevated markers of inflammation.

Recent medical investigation has focused on the identification of novel and more sensitive laboratory markers for disease activity among patients with Takayasu arteritis, with IL-6, IL-18, and certain matrix metalloproteinases showing promise in small studies

What is the treatment of Aortitis?


The treatment of aortitis is dependent on the underlying disorder.

Infectious causes often need antibiotic treatment, while those linked with autoimmune vasculitides are normally treated with steroids.

Treatment involves these treatment priorities:

1. Stop the inflammation,

2. Treat complications,

3. Prevent and monitor for re-occurrence.

Once the diagnosis of aortitis is done, the treatment is dependent upon the underlying cause.

The purposes of treatment are both:

1. The immediate treatment of aortic inflammation, or infection in rare cases, and

2. Monitoring and treatment of aortic and arterial complications.

Infectious Aortitis

In the case of the rare infectious aortitis, the patient requires rapid diagnosis, antibiotic treatment, and consultation with a vascular surgeon.

The initial treatment of suspected infectious aortitis is intravenous antibiotics with broad antimicrobial coverage of the most likely pathologic organisms, mostly staphylococcal species and gram negative rods.

Antibiotics should be started as soon as the diagnosis of infectious aortitis is suspected, and while awaiting microbiologic data.

The antibiotic regime can then be tailored, based upon the results of culture and sensitivity data.

Because of case series documenting a very high mortality rate among patients with gram-negative infectious aortitis treated with medical therapy alone, a combination strategy of intensive antibiotic treatment and surgical debridement, with aneurysm repair if necessary, is normally advised, even though no medical trials have explored the optimal treatment of such patients.

There are small case series, each with no more than a few cases, of combined medical and endovascular treatment of mycotic aneurysm.

In spite of aggressive treatment, mortality linked with infectious aortitis is still high in documented series, mainly because of a high rate of aortic rupture.

While no medical studies have found the optimal period of antibiotic treatment for aortitis, a treatment course of at least 6 to 12 weeks after surgical debridement and clearance of blood cultures is normally advised, with a longer course for patients with immunosuppressive disorders.

Aortitis Linked with Large Vessel Vasculitis

Immunosuppressive medication is the main treatment of non-infectious aortitis occurring from large-vessel vasculitis, and patients are best treated by a multi-disciplinary team that involves a rheumatologist and medical and surgical cardiovascular specialists.

Once the diagnosis of non-infectious aortitis due to GCA or Takayasu arteritis has been made, oral glucocorticoid therapy should be started.

Generally, an initial starting dose of 40-60 milligrams prednisone daily (for GCA) or 1 milligram/kilogram prednisone daily (for Takayasu arteritis) is given, even though the optimal starting dose of steroid therapy for patients with aortitis has not been found.

The prednisone dose is slowly reduced over time with close monitoring of symptoms, vascular signs, inflammatory markers, and imaging studies.

Most patients with arteritis are treated with steroids over a period of months, or even years, in attempt to obtain complete remission.

In spite of glucocorticoid therapy, the recurrence rate for both GCA and Takayasu arteritis is high, up to 50% or greater.

Besides recurrent symptoms, re-elevation of inflammatory markers may be a useful sign of relapsed disease, particularly among patients with GCA.

Because of reduced sensitivity of inflammatory markers for monitoring disease action in Takayasu arteritis, the diagnosis of relapse is more difficult.

The NIH criteria for disease activity in Takayasu arteritis describe active disease as new or worsening of two or more of the following:

1. Systemic symptoms attributable to arteritis,

2. Elevated ESR,

3. Vascular symptoms or examination findings, or

4. Typical angiographic abnormalities.

The part of edema-weighted MRI, 18F-FDG PET, vascular ultrasound, and novel inflammatory biomarkers (e.g., IL-6) in the diagnosis of relapse in Takayasu arteritis are research areas.

Because of the need for long-term therapy and the possibility for steroid-related adverse effects, medicines to prevent osteoporosis, gastric ulceration, and Pneumocystis (carinii) jiroveci pneumonia should be given, as indicated, for patients with aortitis needing long-term glucocorticoid therapy.

Patients should also be carefully reviewed for the formation of steroid-induced diabetes mellitus or hypertension, secondary infections, and cataracts, frequent complication of long-term glucocorticoid therapy.

Patients who have visual symptoms linked to GCA are normally admitted to hospital for intravenous glucocorticoid therapy, to be followed with oral therapy, even though the likelihood of permanent visual deficits stays high, even among those intensively treated.

Patients with severe symptoms linked to aortitis, such as acute aortic insufficiency or coronary artery involvement, should be in the same way treated in hospital with initial intravenous therapy.

Research is been done on the possible steroid-sparing regimes for both GCA and Takayasu arteritis, because of the possible need for long-term immunosuppressive treatment.

Daily administration of prednisone (45 mg daily) was found to be superior to every other day treatment (90mg) in the treatment of GCA.

In recent days, a small trial compared standard oral prednisone therapy to initial induction therapy with intravenous methylprednisolone (15mg/kg) then oral therapy among patients with GCA.

At 78 weeks of follow-up, patients randomized to initial intravenous induction therapy had a lower oral steroid requirement and were less likely to deteriorate, even though there was no significant difference in adverse medical events between the two groups.

3 randomized clinical trials have investigated the possible advantage of the addition of methotrexate to standard oral steroid therapy for the initial treatment of GCA with conflicting findings, and such combination therapy is not normally advised.

A recent small trial of the anti-tumor necrosis factor agent, infliximab, for the maintenance of remission in patients with GCA found no advantage.

Adjunctive, steroid-sparing therapy has also been researched in the treatment of Takayasu arteritis, especially among patients with difficult to treat or relapsing disease.

Methotrexate, azathioprine, mycophenolate mofetil, and infliximab have shown promise.

These agents are a choice for use in steroid-refractory patients, even though data from large randomized medical trials are required to determine their optimal part.

Isolated Idiopathic Aortitis

In rare instances, the active aortitis on histopathology may be determined incidentally in patients with thoracic or abdominal aneurysm who have gone through surgical repair.

Such patients may have no known history of vasculitis and negative systemic signs or symptoms to indicate active or prior disease.

The optimal treatment of such patients is not certain, and the decision to treat with a course of glucocorticoid therapy should be indicated on a case-by-case basis depending on the medical manifestation of the patient and the location and extent of inflammation.

Inflammatory markers, such as the ESR or CRP, will not be reliable during recovery from major aortic surgery, but may be useful in long-term follow-up.

In such patients, imaging of the entire aorta for evidence of active disease or aneurysm in other vascular beds should be indicated.

Patients with isolated idiopathic aortitis require cautious monitoring, as small case series have detected a tendency toward aneurysm formation in other vascular beds over time.

Surgical and Endovascular Therapy

Besides immunosuppressive therapy, patients with aortitis due to large-vessel vasculitis must be closely monitored for the development of vascular complications, particularly evidence of aortic aneurysm or new aortic or arterial occlusive disease.

A comprehensive vascular examination should be performed at each visit.

Measurement of bilateral arm and ankle pressures is useful to assess for arterial occlusive disease, particularly among patients with Takayasu arteritis.

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