Aortitis in Ankylosing Spondylitis

This blog post features recent articles that may offer explanations of the connection between aortitis and Ankylosing Spondylitis.

It’s challenging for doctors and patients to understand what is going on when two conditions present together. Some doctors might attribute the arthritic pain to non-infectious costochondritis (“inflammation of the costal cartilage, the structure that connects each rib to the sternum at the costosternal joint” [Wikipedia: Costochondritis]) and not look further into the deeper vascular phenomena also associated with Ankylosing Spondylitis.

If the aorta is the problem and not the heart itself, will troponin levels be elevated? Not always, according to the case presented by Looi, et al (2011).

In some cases an ECG reveals a problem, and this at least will engage heart specialists.

But what about cases in which the aorta is inflamed in the thoracic or abdominal areas, not near the septum or aortic valve?  What about “periaortitis,” as described below by Palazzi, et al? Will the symptoms of periaortitis likely register as a possibility in the minds of emergency specialists or rheumatologists?

Looi, et al, 2011

LOOI, J. L., PUI, K., HART, H., & EDWARDS, C. (2011). Valvulitis and aortitis associated with ankylosing spondylitis: early detection and monitoring response to therapy using cardiac magnetic resonance imaging. International Journal of Rheumatic Diseases, 14(4), e56–e58. http://doi.org/10.1111/j.1756-185X.2011.01650.x

Dear Editor,
A 44-year-old Pacific Islander with a 10-year history of ankylosing spondylitis (AS) presented with chest heaviness at rest and shortness of breath in addition to inflammatory back pain and secondary osteoarthritis of the right hip.

An electrocardiogram (ECG) showed first-degree atrioventricular (AV) block with a
PR (transit time from sinus node to exit of the AV node) interval of 300 ms. An electrocardiogram in 2003 was normal. Serial troponin I levels were borderline
elevated (maximum 0.04 lg/L, normal < 0.04 lg/L). Inflammatory markers were elevated (erythrocyte sedimentation rate of 22 and C-reactive protein 61 mg/L) in keeping with an acute exacerbation of AS.

Contrast-enhanced cardiovascular magnetic resonance (CE-CMR) was performed to investigate the possibility of myocarditis. This demonstrated striking late enhancement of both aortic and mitral valve annuli, associated with significant soft tissue thickening. (Fig. 1a,b). There was also thickening of the aortic and mitral leaflets, associated with mild mitral regurgitation. The images were consistent with mitral and aortic valvulitis, a non-rheumatic manifestation of acute AS.

[…]

Aortitis, which involves the aortic root and the ascending aorta, leads to valvular insufficiency which rarely occurs in the first decade of disease. Aortitis and valvulitis are rare events and the prevalence is unknown. Valvular changes in AS have been
described as fibrotic, thickened and retracted cusps with rolled edges. These changes lead to aortic insufficiency.

[…]

CMR is a useful imaging modality for detection and quantification of vasculitis in patients with AS. In particular, CMR has greater ability to detect earlier
changes of vasculitis.

Palazzi, et al., 2011

Palazzi, C., Salvarani, C., D’Angelo, S., & Olivieri, I. (2011). Aortitis and periaortitis in ankylosing spondylitis. Joint Bone Spine, 78(5), 451–455. http://doi.org/10.1016/j.jbspin.2010.11.003

ABSTRACT: Aortic involvement is a potential life-threatening complication of ankylosing spondylitis, usually occurring late in the course of this frequent disease.

  • Inflammatory lesions evolving to fibrosis are primarily localized in the aortic root causing regurgitation, but this process can extend into the left atrium (subaortic bump) involving the mitral valve and the heart conduction system.
  • First, second and third degree atrioventricular blocks are the most common conduction alterations described and they can be temporary.

Chronic periaortitis has been described in ankylosing spondylitis patients.

  • This disease is characterized by inflammation evolving to fibrosis and it is localized in the periaortic and peri-iliac retroperitoneum.
  • It causes compressive effects on ureters and venous, arterial and lymphatic vessels.
  • Its treatment employs endoscopic and/or surgical procedures and administration of corticosteroids, even in association with immunosuppressive agents.

Both aortitis (with conduction system alterations) and periaortitis should be kept in mind by the physicians because they can significantly influence the prognosis of ankylosing spondylitis patients and they can need a rapid treatment.

Aortic valve disease (AVD)

  • “The seriousness of the AS-related AVD (aortic valve disease) is highly variable, ranging from chronic and haemodynamically irrelevant fibrosis to acute and rapidly worsening aortic insufficiency.”
  • “The most frequent course is characterized by a slow progression towards symptomatic valvular regurgitation. In fact, a diastolic murmur can be audible for several years before dyspnea develops.” (p. 452)
  • “Transesophageal echocardiography is a better imaging technique to visualize the internal structures of the heart.” Through this method, Roldan, et al. (1998) did a study of 44 AS patients and found:
    • 82% with aortic annulus and valve alterations
    • 61% with aortic root thickening, increased stiffness and dilatation
    • 50% suffered “valvular insufficiency” and 40% with milder lesions.
    • 41% with valvular thickening, mainly of the “nodules of the aortic cusps and basal thickening of the anterior mitral leaflet, as a subaortic bump”
    • 20% showed worsening of the valvular disease with heart failure, valve replacement, stroke or death in comparison with 3% of the control subjects.
    • AS duration was the only feature related to the valvular disease.
  • Conduction abnormalities may range from an asymptomatic course, for first grade atrioventricular blocks or presence of an adequate escape rhythm, to complete heart block causing Stokes-Adam’s attacks requiring urgent hospitalization.” (p. 452)

Chronic Perioaortitis (CP)

  • This category of illness includes 3 syndromes with similar features:
    • RPF (retroperitoneal fibrosis)
    • IAAA (inflammatory abdominal aortic aneurysms)
    • PRF (perianeurysmal retroperitoneal fibrosis), “a combination
      of the first two disorders.”
  • Symptoms:
    • “abdominal, back and/or flank pain, oliguria and uremia,
      constipation, claudication and edema of lower limbs and testicular
      disorders” (453);
    • “Constitutional symptoms and signs such as fever, fatigue, weight loss, anorexia and elevation of ESR and C-reactive protein are common”
  • “The aortic involvement is characterized by inflammation of the peripheral
    layer and degenerative/atherosclerotic lesions in the more internal layers.”
  • IAAA in two reported cases “revealed scattered fibrosis with acute and chronic inflammatory features localized in the perivascular fat. Significant signs of atherosclerosis or calcifications were absent.”
  • “Corticosteroids (CS) and immunosuppressive drugs are commonly used in the management of CP for their action on the inflammatory process with the aim to arrest the damage evolution in abdominal aorta and the progression towards RPF.”
  • “CP can frequently relapse, even several years after a treatment-induced remission. Therefore, the monitoring of inflammation indexes and renal function is required.”
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