Reduced aortic elastic properties in a child with Takayasu arteritis: case report and literature review.
Takayasu arteritis (TA) is a chronic inflammatory vasculitis of the aorta and its major branches with a very low incidence in Europe and North America. Our objective was to determine the elastic properties of the affected ascending and descending aortic walls non-invasively in a 14-year-old Iraqi girl with a 3-year history of fever, fatigue, malaise and diffuse pain. Ultrasound and magnetic resonance angiography showed marked thickening of the aortic wall, dilatation of the aortic arch, and decreased luminal diameters of the abdominal aorta and both subclavian arteries, consistent with TA. Ascending and descending aortic elastic properties such as distensibility and stiffness index were markedly reduced compared to a group of healthy controls (n=39): ascending aortic distensibility was 20 kPa(-1) x 10(-3) versus 63+/-23 kPa(-1) x 10(-3) in controls, and the ascending aortic stiffness index 9.6 versus 3.5+/-1.3 in controls. Although the patient's general condition improved rapidly on oral prednisolone and azathioprine and inflammatory parameters normalised within 3 weeks, the aortic elastic parameters did not change during the first 2 weeks of anti-inflammatory treatment. Unfortunately, no further follow-up was possible.


CONCLUSIONS
In patients with Takayasu arteritis, non-invasive quantification of reduced aortic elastic properties can help to assess aortic involvement, and possibly to follow disease activity and vascular response to therapy.
Baumgartner
Daniela
D
Division of Paediatric Cardiology, Department of Paediatrics, Innsbruck Medical University, Anichstrasse 35, 6020 Innsbruck, Austria. daniela.baumgartner@aon.at
Sailer-Höck
Michaela
M
Baumgartner
Christian
C
Trieb
Thomas
T
Maurer
Heiner
H
Schirmer
Michael
M
Zimmerhackl
Lothar-Bernd
LB
Stein
Jörg-Ingolf
JI
D015746:Abdominal Pain / Q000209:etiology; D000293:Adolescent; D001011:Aorta / Q000187:drug effects / Q000473:pathology* / Q000503:physiopathology; D001379:Azathioprine / Q000627:therapeutic use; D005260:Female; D005334:Fever / Q000209:etiology; D005938:Glucocorticoids / Q000627:therapeutic use; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D018810:Magnetic Resonance Angiography; D008279:Magnetic Resonance Imaging; D011239:Prednisolone / Q000627:therapeutic use; D013625:Takayasu Arteritis / Q000150:complications / Q000175:diagnosis* / Q000188:drug therapy; D016896:Treatment Outcome; D016277:Ventricular Function, Left / Q000187:drug effects
D005938:Glucocorticoids; D007166:Immunosuppressive Agents; D011239:Prednisolone; D001379:Azathioprine