Aortitis is the

inflammation Inflammation (from ) is part of the biological response of body tissues to harmful stimuli, such as pathogens, damaged cells, or irritants. The five cardinal signs are heat, pain, redness, swelling, and loss of function (Latin ''calor'', '' ...

of the aortic wall. The disorder is potentially life-threatening and rare. It is reported that there are only 1–3 new cases of aortitis per year per million people in the United States and Europe. Aortitis is most common in people 10 to 40 years of age.

Causes

This

has a number of possible causes, including trauma, viral or bacterial infections (notably,

syphilis Syphilis () is a sexually transmitted infection caused by the bacterium ''Treponema pallidum'' subspecies ''pallidum''. The signs and symptoms depend on the stage it presents: primary, secondary, latent syphilis, latent or tertiary. The prim ...

), and certain immune disorders and connective tissue diseases. Aortitis is most commonly seen in patients with

, autoimmune vasculitis (

giant cell arteritis Giant cell arteritis (GCA), also called temporal arteritis, is an inflammatory autoimmune disease of large blood vessels. Symptoms may include headache, pain over the temples, flu-like symptoms, double vision, and difficulty opening the mout ...

Takayasu's arteritis Takayasu's arteritis (TA), also known as aortic arch syndrome, nonspecific aortoarteritis, and pulseless disease, is a form of large vessel granulomatous vasculitisAmerican College of Physicians (ACP). Medical Knowledge Self-Assessment Program (M ...

polymyalgia rheumatica Polymyalgia rheumatica (PMR) is a systemic inflammatory disease characterized by pain or stiffness, usually in the neck, shoulders, upper arms, and hips, but which may occur all over the body. Almost all cases occur in people age 50 or older. ...

, and

rheumatoid arthritis Rheumatoid arthritis (RA) is a long-term autoimmune disorder that primarily affects synovial joint, joints. It typically results in warm, swollen, and painful joints. Pain and stiffness often worsen following rest. Most commonly, the wrist and h ...

. aber's Cyclopedic Medical Dictionary. 19th ed. Philadelphia: F.A. Davis, 2001./ref> IgG4-related disease has more recently been identified as a cause of aortitis, and also as a cause of periaortitis (inflammation ''surrounding'' the aorta). There is a wide range of symptoms that are dependent on the location of the aortic inflammation or associated disorder. A few symptoms recognized in patients are

fever Fever or pyrexia in humans is a symptom of an anti-infection defense mechanism that appears with Human body temperature, body temperature exceeding the normal range caused by an increase in the body's temperature Human body temperature#Fever, s ...

, chills, muscle pains, and malaise (general discomfort). In addition,

hypertension Hypertension, also known as high blood pressure, is a Chronic condition, long-term Disease, medical condition in which the blood pressure in the artery, arteries is persistently elevated. High blood pressure usually does not cause symptoms i ...

(high blood pressure) may occur. Hypertension happens when the renal artery narrows and elasticity of the aorta and branches decrease.Link text
American Heart Association. Aortitis can also be caused by ''

Salmonella ''Salmonella'' is a genus of bacillus (shape), rod-shaped, (bacillus) Gram-negative bacteria of the family Enterobacteriaceae. The two known species of ''Salmonella'' are ''Salmonella enterica'' and ''Salmonella bongori''. ''S. enterica'' ...

'' infection.

Diagnosis

Diagnosis of aortitis starts with

physical examination In a physical examination, medical examination, clinical examination, or medical checkup, a medical practitioner examines a patient for any possible medical signs or symptoms of a Disease, medical condition. It generally consists of a series of ...

and recording patient's medical history. Imaging tests such as

magnetic resonance angiography Magnetic resonance angiography (MRA) is a group of techniques based on magnetic resonance imaging (MRI) to image blood vessels. Magnetic resonance angiography is used to generate images of arteries (and less commonly veins) in order to evaluate ...

doppler ultrasonography Doppler ultrasonography is medical ultrasonography that employs the Doppler effect to perform imaging of the movement of tissues and body fluids (usually blood), and their relative velocity to the probe. By calculating the frequency shift of a ...

, and

positron emission tomography Positron emission tomography (PET) is a functional imaging technique that uses radioactive substances known as radiotracers to visualize and measure changes in metabolic processes, and in other physiological activities including blood flow, r ...

(PET) can be used to look at the blood vessels and blood flow, and highlight areas of inflammation. Condition is generally diagnosed based on the symptoms the patient is experiencing and the aortic images.

Treatment

Treatment of aortitis depends on the underlying cause. The initial treatment of suspected infectious aortitis is intravenous antibiotics with broad antimicrobial coverage of the most likely pathologic organisms, In contrast, immunosuppressive therapy is the primary treatment of non-infectious aortitis due to large-vessel vasculitis, and patients are ideally managed by a multi-disciplinary team that includes a rheumatologist and medical and surgical cardiovascular specialists. Once the diagnosis of non-infectious aortitis due to GCA or Takayasu arteritis has been established, oral glucocorticoid therapy should be initiated. Frequently, adjunctive immunosuppressants, including

methotrexate Methotrexate, formerly known as amethopterin, is a chemotherapy agent and immunosuppressive drug, immune-system suppressant. It is used to treat cancer, autoimmune diseases, and ectopic pregnancy, ectopic pregnancies. Types of cancers it is u ...

and azathioprine, are paired with steroids to treat refractory disease or to minimize steroid-induced adverse effects. Management includes the following treatment priorities: stop the

, treat complications, prevent and monitor for re-occurrence.

Prognosis

If untreated, has three distinct phases. The first is a prepulseless inflammatory stage with nonspecific symptoms such as fatigue, arthralgias, and low-grade fevers. Phase two includes vascular inflammation with pain secondary to the condition, along with tenderness to palpation over the site. The last phase includes symptoms of

ischemia Ischemia or ischaemia is a restriction in blood supply to any tissue, muscle group, or organ of the body, causing a shortage of oxygen that is needed for cellular metabolism (to keep tissue alive). Ischemia is generally caused by problems ...

and pain associated with the use of limbs. Limbs are also cool and clammy in this stage.

References

External links

{{Vascular diseases Vascular diseases IgG4-related disease