What are the other Names for this Condition? (Also known as/Synonyms)

• Inflammation of the Aorta
• Inflammation of the Aortic Wall
• Inflammatory Aortic Disease

What is Aortitis? (Definition/Background Information)

• Aortitis is a rare form of vasculitis, which are a group of disorders that result in the inflammation of the blood vessels. When inflammation of the blood vessels occurs, it causes thickening of the blood vessel walls, thus narrowing the arteries supplying blood and oxygen to the various parts of the body/organ. This can result in organ dysfunction
• Aortitis is described as inflammation of the aorta; the aorta is the largest artery in the body that supplies oxygenated blood from heart to the rest of the body. In many cases, Aortitis occurs against a background of certain inflammatory disorders and infections. In rare cases, Aortitis can occur without a clearly identifiable cause
• The common infections associated with Aortitis include hepatitis B and C viral infections, tuberculosis, and syphilis, among others; while, the non-infectious conditions causing Aortitis include rheumatic diseases such as lupus, ankylosing spondylitis, and rheumatoid arthritis
• Aortitis can affect many different organs in the body, including the muscles and joints and heart, causing a variety of signs and symptoms, such as fever, fatigue, weight loss, muscle and joint pain, abdominal pain, and dizziness
• In order to establish a diagnosis of Aortitis, a wide variety of tests and exams may be performed that include blood and urine tests, radiological studies such as Doppler ultrasound and an echocardiogram, including a tissue biopsy, when needed
• An early diagnosis and appropriate treatment of Aortitis, using corticosteroid therapy and immunosuppressive medications, as need, can lead to a good prognosis. However, a late recognition and delayed management of the condition can severely worsen the outcomes

Who gets Aortitis? (Age and Sex Distribution)

• Aortitis is an uncommon condition that can be generally seen across a wide age group affecting both males and females
• Although, most cases are observed in children and young adults in the 10 to 40 years’ age range
• It is observed worldwide and can affect people of all racial and ethnic backgrounds
• The incidence rate of the condition per year in the US and UK is reportedly 1-3 cases per million population

What are the Risk Factors for Aortitis? (Predisposing Factors)

In many individuals, the risk factors for Aortitis are not clearly established. It may be associated with certain infectious and non-infectious conditions (such as autoimmune disorders). The risk factors for Aortitis may include:

• Infectious conditions such as:
  • Hepatitis B and hepatitis C infections
  • Herpes virus infection
  • Rocky Mountain spotted fever
  • Salmonellosis
  • Syphilis
  • Tuberculosis
• Non-infectious conditions such as:
  • Ankylosing spondylitis
  • Rheumatoid arthritis (RA)
  • Sarcoidosis
  • Systemic lupus erythematosus (SLE)

It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases one’s chances of getting a condition compared to an individual without the risk factors. Some risk factors are more important than others.

Also, not having a risk factor does not mean that an individual will not get the condition. It is always important to discuss the effect of risk factors with your healthcare provider.

What are the Causes of Aortitis? (Etiology)

Aortitis is the inflammation of an aorta, which is a large blood vessel of the body. The condition is a form of large vessel vasculitis; and, other small and medium-sized blood vessels are usually not affected.

• The large blood vessel vasculitis types associated commonly with Aortitis are giant cell arteritis (GCA) and Takayasu arteritis; these two conditions may be described as the most common causes of Aortitis
• Other vasculitis types associated with it include Bechet’s disease, Cogan's syndrome, and other ANCA-associated vasculitis

The cause of Aortitis may be categorized into the following three sections:

• Non-Infectious Aortitis causes, which are inflammatory disorders that affect the joints and muscles leading to pain and inflammation. Such disorders include:
  • Rheumatoid arthritis (RA)
  • Sarcoidosis
  • Systemic lupus erythematosus (SLE)
  • HLA-B27 associated spondyloarthropathies such as ankylosing spondylitis and Reiter syndrome
  • Relapsing polychondritis
  • Certain connective tissue disorders
• Infectious Aortitis causes, which are several of the bacterial and viral infections affecting the body. Such infections include:
  • Hepatitis B and C viral infections
  • Herpes virus infection
  • Rocky Mountain spotted fever
  • Salmonella infection 
  • Syphilis
  • Tuberculosis
• Isolated Aortitis, wherein the inflammation of aorta occurs in the absence of any identifiable or underlying cause

In many cases, Aortitis is believed to be a hypersensitivity reaction to certain stimuli triggering an abnormal immune reaction. Such stimuli could be in the form of inflammatory disorders, infections, or various other factors.

What are the Signs and Symptoms of Aortitis?

The severity of signs and symptoms of Aortitis may vary from one individual to another. In some, it may be mild, resulting in a delayed diagnosis of the condition. In other individuals, it may be severe. The signs and symptoms of Aortitis mainly depend on the underlying cause and the organs affected. In many individuals, the involvement of the heart is commonly noted.

The signs and symptoms of Aortitis may include:

• Fever
• Headaches
• Fatigue
• Loss of weight
• Weakness
• Pain that may affect the chest, back, or abdomen
• Shortness of breath
• Vision problems

Based on the involvement of the various organs/systems, the following signs and symptoms may be noted. It is important to note that the combination of signs and symptoms varies from individual to individual.

Signs and symptoms related to the muscles and joints (musculoskeletal system):

• Inflammation of the muscles and joints
• Muscle and joint pain, involving one or more joints
• Tenderness of the muscles
• Muscle stiffness and spasms
• Swollen joints
• Joint stiffness
• Muscle weakness, especially involving the upper or lower limbs
• Loss of muscle mass
• Muscle contracture
• Difficulty climbing stairs and walking
• Difficulty with other motor skills, including jumping, hopping, and running
• Difficulty in bending or stretching one’s back
• Limited range of motion; decreased joint function
• The condition may involve any joint in the body such as the knee, elbow, shoulder, hip, etc.

If the heart is involved, the signs and symptoms related to the heart and blood vessels (cardiovascular system) that may be additionally noted include:

• Breathing difficulties
• Sweating and discomfort
• Heart palpitations
• Blood in sputum
• Confusion
• Abnormal heartbeats (arrhythmias)
• Dizziness or lightheadedness
• Fainting (syncope)
• Cold and clammy hands and feet indicate an advanced stage of the condition

How is Aortitis Diagnosed?

A diagnosis of Aortitis usually depend on the underlying cause and the system or organ of the body that is affected. The diagnosis may involve the following tests and examinations:

• Complete physical examination and a thorough medical history
• Assessment of the presenting signs and symptoms

Tests and procedures based on the different body part/organ or system that is affected may include any of the following.

• Blood and urine tests related to overall inflammatory and autoimmune activity:
  • Complete blood count (CBC) with differential: This measures the red blood cell count and hemoglobin levels in blood
  • Erythrocyte sedimentation rate (ESR): ESR is a blood test that can help the healthcare provider identify any inflammatory activity within the body
  • C-reactive protein (CRP) test: This blood test measures the amount of a protein, called C-reactive protein, in blood. It also provides information on the inflammation condition of the body
  • Anti-nuclear antibody (ANA) test: ANA test identifies the antinuclear antibodies within the blood
  • Basic metabolic panel tests
  • Anti-streptolysin O (ASO) titer test, if a streptococcal infection is the suspected trigger
  • Specific antibodies test
  • Protein electrophoresis and immunofixation electrophoresis tests
  • Complement blood test
  • Cryoglobulin blood test
  • HIV antibody blood test
  • Urine tests for protein and presence of blood
  • Urinalysis

Tests and procedures related to the muscles and joints (musculoskeletal system):

• Laboratory tests, which may include:
  • Creatine kinase test: It is used to measure the amount of creatine kinase in blood. The enzyme creatine kinase is released by the muscle and into the bloodstream, when any deterioration has occurred
  • Rheumatoid factor blood test
  • Cyclic citrullinated peptide antibody blood test (anti-CCP)
• Electromyogram (EMG): Electromyographic studies to determine the region of muscle that is affected
• Radiological imaging studies of the affected region such as:
  • X-ray studies
  • CT scans
  • MRI scans
  • Ultrasound imaging: An ultrasound imaging equipment uses high-frequency sound waves to generate a more detailed image of affected region
  • Musculoskeletal angiographic studies
• Fine needle aspiration (FNA) of joint fluid: Occasionally, fluid may accumulate around a joint, which results in pain and disability. Analysis of this joint will provide clues regarding the cause of joint fluid accumulation
• Muscle biopsy: A biopsy of the affected muscle tissue is performed and sent to a laboratory for a pathological examination. A pathologist examines the biopsy sample under a microscope. After putting together clinical findings, special studies on tissues (if needed) and with microscope findings, the pathologist arrives at a definitive diagnosis. Examination of the biopsy sample under a microscope by a pathologist is considered to be gold standard in arriving at a conclusive diagnosis

Tests and procedures related to the heart and blood vessels (cardiovascular system):

• Electrocardiogram (EKG or ECG): It is used to measure the electrical activity of the heart to detect arrhythmias
• Electrophysiological studies of the heart, to determine where arrhythmia is getting generated in the heart, is often helpful
• Checking oxygen levels in blood through the skin using a pulse oximeter
• Checking oxygen levels in blood using an arterial blood gas test
• Radiological imaging studies that include:
  • CT scan of the thorax
  • MRI scan of the heart
  • Echocardiography: This procedure uses sound waves to create a motion picture of the heart movement. It can help show the size and shape of the heart and how well the chambers and valves are working
  • Doppler ultrasound: Sound waves are used to measure the speed and direction of blood flow
• Cardiac catheterization: The passage of a thin catheter into the right or left side of the heart to diagnose (and may be treat) cardiovascular conditions
• Tissue biopsy:
  • A tissue biopsy is performed and sent to a laboratory for a pathological examination. A pathologist examines the biopsy under a microscope. After putting together clinical findings, special studies on tissues (if needed) and with microscope findings, the pathologist arrives at a definitive diagnosis. Examination of the biopsy under a microscope by a pathologist is considered to be gold standard in arriving at a conclusive diagnosis
  • Biopsy specimens are studied initially using Hematoxylin and Eosin staining. The pathologist then decides on additional studies depending on the clinical situation
  • Sometimes, the pathologist may perform special studies, which may include immunohistochemical stains, molecular testing, and very rarely, electron microscopic studies to assist in the diagnosis

Other diagnostic examinations may be performed based on the affected region/part of the body and the nature of the underlying condition.

Many clinical conditions may have similar signs and symptoms. Your healthcare provider may perform additional tests to rule out other clinical conditions to arrive at a definitive diagnosis.

What are the possible Complications of Aortitis?

The complications of Aortitis that develop depend on the underlying cause, the organs affected, severity of the signs and symptoms, and one’s response to treatment.

Complications associated with the muscles and joints (musculoskeletal system) may include:

• Chronic pain and weakness of the joint affecting the quality of life
• Formation of scar tissue in the muscle and joint tissue
• Immobilization due to loss of movement
• Permanent disability

Complications associated with the heart and blood vessels (cardiovascular system) may include:

• Severe arrhythmias
• Normal function of the heart is severely affected
• Aortic insufficiency, due to incomplete valve closure
• It can weaken of the aorta causing aortic aneurysm, which is a potentially life-threatening complication
• Congestive heart failure
• Increased risk for thromboembolism (blood clot obstructing a blood vessel)
• Chronic high blood pressure in the blood vessels of the lungs

Treatment complications: Some complications are known to take place with treatment; in some cases, due to treatment also. Immunosuppressive therapy that is used to treat Aortitis may lead to certain complications that include:

• Low blood pressure
• Hyperkalemia or high levels of potassium in blood
• Tremors
• Diabetes mellitus
• Osteoporosis causing decreased bone density
• Decreased muscle function
• Long-term kidney dysfunction
• Elevated risk for malignancy
• Decrease in body immunity to fight infections

How is Aortitis Treated?

The treatment of Aortitis depends upon a variety of factors including:

• Severity of the signs and symptoms
• Regions of the body affected and the level of functioning of the affected organs
• Overall health status of the individual and his/her age
• Underlying cause: If it is caused by an underlying condition, then treating the same improves the symptoms associated with it

The main treatment of Aortitis includes treating the underlying cause. If a clearly identifiable cause is not known, then the primary role of treatment is to reduce inflammation. Often, a multidisciplinary team of specialists that include general practitioners, rheumatologists, neurologists, pulmonologists, cardiovascular experts, nephrologists, dermatologists, ophthalmologists, among others, may be involved in the treatment.

The treatment methods for the condition may include:

• Most cases are thought to arise as a result of hypersensitivity to an offending stimulus, hence removal of the stimulus is often sufficient treatment for the illness
• Corticosteroid therapy
• Immunosuppressive medications
• Biologic agents using specific monoclonal antibodies to reduce inflammation
• In cases with severe symptoms, or when the affected individuals do not respond to the above-mentioned treatments, plasma exchange (plasmapheresis) or intravenous immunoglobulin (IVIG) therapy may be considered
• Rarely, vascular surgery, such as vascular bypass procedures, vascular stent placements, and removal of blood clots (thrombectomy), are found to be beneficial
• If the heart is severely affected, then the following invasive procedures may be considered:
  • Bypass surgery for large arterial blockage
  • Repair or replacement of heart valve
  • Percutaneous angioplasty for severe blocks
  • Aortic aneurysmal repair, if needed
• Follow-up care with regular screening and check-ups are important and encouraged

The treatment also depends on whether there are recurring episodes of the condition. The goal of the treatment is to control the inflammation in order to achieve remission (a state where no signs and symptoms are noted) and maintenance therapy to prevent episodes of relapses.

How can Aortitis be Prevented?

Currently, there are no methods available to prevent Aortitis if the cause is unknown. An early diagnosis and prompt treatment can help decrease the burden of the condition.

• Undertaking early and appropriate treatment of the underlying infection or inflammatory disorder may help in lowering one’s risk for the condition
• Managing the underlying infection or inflammatory condition that triggered the initial episode helps in preventing subsequent episodes of vasculitis
• Maintaining a healthy lifestyle with good eating habits, adequate physical activities, and avoidance of smoking or drinking is beneficial
• Ensuring that individuals, especially children, are up-to-date on their vaccinations is important
• Patients are required to be vigilant during the remission period and report any new symptoms promptly to the healthcare provider

Regular medical screening at periodic intervals with blood tests, scans, and physical examinations, are mandatory, due to the possibility of recurrence/relapse. Often several years of active vigilance is necessary.

What is the Prognosis of Aortitis? (Outcomes/Resolutions)

• The prognosis of Aortitis depends upon a variety of factors including the:
  • Severity of Aortitis; degree of damage to the vital organs and body systems
  • Age and overall health of the individual
  • One’s response to treatment: Individuals who respond to treatment usually have a better prognosis than those who do not respond well to treatment
  • The underlying condition that triggered Aortitis
  • Whether it is an initial episode or a recurring episode
• Aortitis affects the heart and aorta. Individuals with extensive condition affecting several regions of the body have poorer prognosis, than individuals in whom the condition is confined to certain part(s) of the body
• The inflammation in Aortitis can be either short-term or long-term, leading to the development of acute or chronic conditions, respectively. Constant medical surveillance and imaging scans are important to prevent adversities
• With early diagnosis and removal of the offending agent/underlying condition, the prognosis is good for many individuals. If left untreated, the condition can potentially cause significant tissue injury, organ failure, or even death

Even with treatment, Aortitis can relapse. If blood in cough or blood in urine is observed, then it is extremely important to contact the healthcare provider right away.

Additional and Relevant Useful Information for Aortitis:

The following article link will help you understand other Vein & Vascular Health:

https://www.dovemed.com/health-topics/vein-and-vascular-center/