Idiopathic Inflammatory Ascending Aortitis Treated Successfully with Steroids

Article Citation:

Matthew C. Chu, William P. Dickey, Frazier Eales, Benjamin K. Johnson, Luis Pagan-Carlo, and Nedaa Skeik (2017) Idiopathic Inflammatory Ascending Aortitis Treated Successfully with Steroids. Journal of the Minneapolis Heart Institute Foundation: Fall/Winter 2017, Vol. 1, No. 2, pp. 145-148.

Case Report

Matthew C. Chu, MD

William P. Dickey, MD

Frazier Eales, MD

Benjamin K. Johnson, MD

Luis Pagan-Carlo, MD

Nedaa Skeik, MD

Minneapolis Heart Institute Foundation
Abbott Northwestern Hospital
Minneapolis, MN

Address for correspondence: 
Matthew C. Chu, MD
Minneapolis Heart Institute®
800 East 28th Street
Minneapolis, MN 55407

E-mail: matthew.chu@allina.com

ABSTRACT

Patients with aortitis, which can be infectious or inflammatory, may have findings that mimic the acute aortic pathology from an aortic dissection, intramural hematoma, or a penetrating aortic ulcer. A prompt determination of the etiology of aortic pathology via multimodal imaging and clinical assessment is critical to guiding care because treatment for each condition varies widely.

Keywords: inflammatory aortitis, noninfectious aortitis, aortic dissection, aortic aneurysm, intramural hematoma

INTRODUCTION

Aortitis can be difficult to distinguish from other forms of acute aortic disease, including aortic dissection, penetrating aortic ulcer, and intramural hematoma.1–3 Immediate surgical repair is often required for the latter conditions but the prognosis is poor when surgery is performed on a patient with uncontrolled aortitis.4 A prompt and accurate diagnosis is possible when using the full array of available multimodal imaging combined with clinical assessment. Aortitis may be infectious in nature in which case it is treated with a combination of antimicrobials and surgery.5–7 Alternatively, aortitis may be inflammatory (noninfectious) and require steroids and occasionally immune-modulators. A significant number of patients with inflammatory aortitis also have an aneurysm that will require surgical repair once the aortic inflammation has been controlled.8

CASE PRESENTATION

A 50-year-old male with tobacco use and obesity presented with 6 days of retrosternal chest pain. This was sharp in nature, moderate in intensity, and waxed and waned but never resolved entirely. It was both pleuritic and positional, as it improved with sitting upright. There was also shortness of breath and lower extremity edema.

The patient described feeling generally unwell for 2 weeks experiencing intermittent fevers, chills, sweats, a general sense of malaise and nonspecific abdominal pain. He had recently been seen by a dentist for oral pain and was treated with clindamycin with partial relief. Review of systems revealed a petechial rash on the extremities. Family history was positive for an aortic dissection in a half-brother.

He presented to an outside hospital where a pulmonary artery CT angiogram did not show a pulmonary embolism. However, there was a large pericardial effusion, mild mediastinal lymphadenopathy, and a 5.9-cm ascending thoracic aortic aneurysm with irregular thickening. He was transferred emergently to Abbott Northwestern Hospital due to a concern for acute aortic pathology possibly requiring immediate cardiothoracic surgical intervention.

Upon arrival at Abbott Northwestern Hospital, he had atrial flutter with a heart rate of 134 beats per minute and a blood pressure of 142/91. A transthoracic echocardiogram (TTE) showed a pericardial effusion with compression of the right ventricle and an enlarged aorta (Figure 1). A transesophageal echocardiogram (TEE) confirmed circumferential thickening of the aortic root and proximal ascending aortic wall with an associated aneurysm suspicious for an intramural hematoma versus aortic dissection (Figure 2). A gated chest CT was timed to opacify the aorta. This ruled out an aortic dissection but confirmed a thickened aortic root with an aortic aneurysm measuring 6.5 cm (Figure 3). A positron emission tomography (PET) scan showed inflammation of the ascending aorta in the region of the aortic aneurysm (Figure 4).

FIGURE 1
Subcostal view of a TTE showing a moderate to large pericardial effusion with compression of the right ventricle (arrow) concerning for tamponade.

FIGURE 2
High esophageal view of a TEE showing an aneurysmal aorta with a thickened wall concerning for possible intramural hematoma versus dissection (arrow).

FIGURE 3
Nongated CT angiogram showing an aneurysmal ascending aorta with associated wall thickening (small arrow) at the level of the pulmonary artery. There is an associated inflamed pericardial reflection (large arrow) with fluid.

FIGURE 4
PET-FDG scan showing diffuse uptake (arrow) in the ascending aorta in the region of aneurysm consistent with active inflammation.

Additional laboratory values were as follows: white blood count of 16,600/mm3, hemoglobin of 11.1 g/dL, platelets of 496,000/mm3, troponin I of less than 0.10 ng/mL, C-reactive protein of 24 mg/dL, and a sedimentation rate of 96 mm/hour. Rheumatologic workup showed a mildly elevated antinuclear antibody ratio that was speckled at 1:80, antineutrophil cytoplasmic antibodies that were positive at greater than or equal to 1:160, and rheumatoid factor of 33.3 IU/mL. Blood cultures, a TB test (QuantiFERON-TB gold; Qiagen, Germantown, MD) and the Treponema pallidum test were all negative.

The diagnosis of idiopathic inflammatory aortitis was based on the multimodality imaging, which showed an aortic aneurysm with diffuse wall thickening and fluorine-18-fluorodeoxyglucose (FDG) uptake consistent with an inflammatory process. The laboratory markers were consistent with an inflammatory state and suggestive of a possible underlying rheumatologic condition.

Originally, the patient had been transferred to this tertiary facility with a tentative plan of surgery for acute aortic pathology. Due to the evidence of an acute inflammatory process without intramural hematoma or dissection, the treatment plan was altered to focus on medical treatment for acute inflammatory aortitis. The patient was placed on high-dose steroids. A chemical cardioversion with ibutilide was performed with return to sinus rhythm. An echocardiogram guided pericardiocentesis was scheduled 2 days after initiating steroid treatment but was canceled because an intraprocedure echocardiogram showed significant resolution of the pericardial effusion (Figure 5). He was discharged from the hospital with a plan for surgical intervention once his acute inflammatory process stabilized.

FIGURE 5
Intra-procedural subcostal TTE shows a marked improvement in the size of the pericardial effusion (arrow) following 48 hours of steroid treatment.

DISCUSSION

Aortitis can easily be confused with an acute aortic syndrome such as aortic dissection, penetrating ulcer and intramural hematoma.9,10 Inflammatory changes to the aortic wall are characteristic of aortitis and may be from an infectious or noninfectious origin. Because of the life-threatening potential of an acute aortic pathology and the markedly different therapy for aortitis, it is critical to accurately make the diagnosis.

Multiple complementary imaging modalities are available both for diagnosis and long-term follow-up of thoracic aortic disease.11

Echocardiography has the advantage of assessing at the bedside both the aorta and the aortic valve as well as the other elements of cardiac structure and function. TTE is noninvasive and often used to assess undifferentiated chest pain. Besides providing information about the aortic dimensions and thickness, it can detect evidence of left- and right-sided heart failure, pericardial effusion, myocardial infarction, valvular heart disease, and pulmonary hypertension. TEE is a useful adjunct to TTE because it provides improved visualization of both the ascending and descending thoracic aorta with better spatial resolution. It is particularly helpful in examining for evidence of vegetation or pseudoaneurysm that can accompany infectious aortitis.12 A shortcoming of TEE is that the aortic arch is difficult to visualize as the ultrasound waves are obstructed by air in the trachea. TEE is often used to assist with perioperative evaluation of the aorta and aortic valve.

PET scan localize inflammatory changes in the vasculature as FDG accumulates in monocytes but not in normal vasculature. Fluorine-18-fluorodeoxyglucose uptake can be seen in both aortitis and atheromatous disease.13 This modality is the most sensitive test for diagnosing early or developing aortitis. PET imaging has been used in some cases to track disease progression and treatment response.13,14

Computed tomography (CT) is the most frequently used test in the emergency department to examine for acute aortic pathology as well as pulmonary embolism. Gated CT allows for more exact assessment of the aortic root, which can be blurred by cardiac motion on nongated studies, leading to significant artifacts that can mimic aortic dissection. Contrast is used to opacify both the arteries and veins, but can increase risk of acute kidney injury related to contrast nephropathy. Assessment of the Hounsfield units of the aortic wall on both noncontrast and contrast images can help differentiate aortic hematoma from aortits.11 CT offers excellent spatial resolution, making it highly sensitive at detecting acute aortic pathology and allowing for detailed characterization of the mural thickening present in aortitis.15,16

Treatment of inflammatory aortitis has not been studied in a randomized controlled fashion. To date, retrospective chart and pathology reviews as well as disease registries constitute the bulk of the published literature.17–21 Infectious aortitis is less common and may require both antimicrobial therapy as well as surgical debridement and resection.4 For inflammatory aortitis, initial treatment generally consists of steroids and immune-modulators. Once the inflammation is controlled, patients may proceed to surgery if there is a standard surgical indication such as a significant aneurysm.10Possible complications of aortitis include aortic dissection and rupture, which require emergent surgical intervention.

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