Teaching Case

A 70-year-old woman was hospitalised for diagnostic workup because of long-term coughing without haemoptysis. HRCT shows pulmonary consolidation in the left lung. Despite the proper treatment of pneumonia, the patient complained about increasing fatigue, general malaise. Due to unintentional weight loss, PET/CT scanning was requested to rule out malignancy.

PET scan:

Normal FDG uptake in the lungs and mediastinum. Moderate to severe increase of FDG uptake in the aortic wall shows wall thickening in the thorax and abdomen.

CT scan:

Ascending aorta measured up to 4 cm. Abdominal and thoracic aortic wall is uninterruptedly oedematous and thickened, which implies inflammation in the aorta. No malignancy-suspected changes were detected in the liver, spleen, adrenal glands, pancreas, kidneys and spinal column. No lymphadenopathy.

Conclusion:

Oedematous and thickened aortic wall throughout the abdomen and thorax with moderate to severe increase of FDG uptake indicating Panaortitis. No changes indicating malignancy were detected.

Background:
Aortitis is an inflammation of the aortic wall. This disorder is potentially life-threatening and rare. There are only 1–3 new cases of aortitis per year per million in the United States and Europe. [1] It is rarely seen in individuals younger than 40 years of age. Most of the patients are older than 65 years. Aortitis often leads to a dilatation of the aortic root and a secondary aortic insufficiency. Aortic wall inflammation may be infectious or more commonly noninfectious. Noninfectious aortitis occurs in large-vessel vasculitis such as Takayasu arteritis and giant cell arteritis (GCA). [2, 4] It is also seen in other collagen vascular disorders such as rheumatoid arthritis and ankylosing spondylitis. [5] The aorta is normally very resistant to infection, but it can be vulnerable because of surgery, aneurysm and systemic disease. Infectious aortitis may be secondary to tuberculosis, syphilis or other pathogens. Aortitis can also occur idiopathically or radiation-induced. [2, 4]
Clinical Perspective:
The clinical manifestations are usually vague and nonspecific and may include pain, fever, vascular insufficiency [2, 3], and elevated levels of acute phase reactants, as well as other systemic manifestations. [4]
Imaging Perspective:
CT angiography, MR imaging, position emission tomography scans and/or confirmation by biopsy play a role in evaluation and further assessment. [5] CT angiography is essential in work-up of aortitis because it allows early diagnosis. Findings include concentric thickening of the vessel wall, thrombosis, stenosis, and occlusion. Other associated findings include vessel ectasia, aneurysms, and ulcers. Aortic wall thickening, which has been described as a “double ring” appearance at contrast-enhanced CT, is the typical finding in the early stage, with a poorly enhanced internal ring (the swollen intima) and an enhancing outer ring (the inflamed media and adventitia). CT angiography has high sensitivity and specificity (95% and 100%, respectively) for demonstrating the abnormalities of the affected vessels and is better than conventional angiography in demonstrating wall thickening, calcification, and mural thrombi. Transoesophageal echocardiography and intravascular US are important tools that provide high-resolution images of subtle changes in aortic segments. MR imaging demonstrates early wall thickening even before luminal narrowing occurs. Gadolinium-enhancement and different sequences are used to ease the diagnosing. PET images can show increased uptake in the periaortic tissue. [2] In this case, diagnosis was performed by PET CT scan, which was consistent with clinical and laboratory findings, so there is no MRI in the patient’s profile.
Outcome:
Aortitis is often overlooked during the initial work-up of patients with systemic disorders. The incidence of this disease is higher among women. [3]

Take Home Message
Think of rare diseases, or else you cannot find them.

Written informed patient consent for publication has been obtained.