Specialty cardiology ICD-9-CM 447.6 MeSH D001025 | ICD-10 I77.6 eMedicine med/158 | |
Aortitis is the inflammation 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.
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Causes
This inflammation has a number of possible causes, including trauma, viral or bacterial infections (notably, syphilis), and certain immune disorders and connective tissue diseases.
Aortitis is most commonly seen in patients with syphilis, autoimmune vasculitis (giant cell arteritis, Takayasu's arteritis), polymyalgia rheumatica, and rheumatoid arthritis. IgG4-related disease is a more recently identified cause of aortitis and periaortitis.
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, chills, muscle pains, and malaise (general discomfort). In addition, hypertension (high blood pressure) may occur. Hypertension happens when the renal artery narrows and elasticity of the aorta and branches decrease.
Treatment
Treatment of aortitis depends on the underlying cause. Infectious causes commonly require antibiotic treatment, while those associated with autoimmune vasculitides are generally treated with steroids.
Management includes the following treatment priorities: stop the inflammation, 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 and pain associated with the use of limbs. Limbs are also cool and clammy in this stage.