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IMAGES IN RHEUMATOLOGY
Ahead of print publication  

Imaging of takayasu arteritis with renal infarct


 Department of Radio-Diagnosis, Sree Balaji Medical College and Hospital, Chennai, Tamil Nadu, India

Date of Submission29-Jul-2020
Date of Acceptance19-Aug-2020

Correspondence Address:
Venkatraman Indiran,
Department of Radio.Diagnosis, Sree Balaji Medical College and Hospital, 7 Works Road, Chromepet, Chennai - 600 044, Tamil Nadu
India
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/injr.injr_206_20

  Abstract 


Keywords: Arteritis, infarct, Takayasu



How to cite this URL:
Kanase ND, Ethiraj D, Indiran V. Imaging of takayasu arteritis with renal infarct. Indian J Rheumatol [Epub ahead of print] [cited 2020 Oct 27]. Available from: https://www.indianjrheumatol.com/preprintarticle.asp?id=298049




  Case Report Top


A 9-year-old female child presented to our hospital with chief complaints of severe headache and upper abdominal pain for 2 months. Her blood pressure readings were 170/110 mm of Hg (right upper limb), 166/106 mm of Hg (left upper limb), 162/102 mm of Hg (right lower limb), and 164/106 mm of Hg (left lower limb). Auscultation revealed pan-systolic murmur over the precordium. Abdominal and bilateral carotids did not show any abnormal vascular bruits. Blood investigations revealed elevated erythrocyte sedimentation rate of 75 mm/h. Computed tomography angiography (CTA) revealed ~50%–60% narrowing of the descending aorta for ~ 2–3 cm and ~50% stenosis of the left renal artery with a hypodense infarct in the lower pole of ipsilateral kidney [Figure 1] and [Figure 2]. The acute phase of Takayasu arteritis (TA) is a granulomatous infiltrative process where elastic fibers of the tunica media are involved. This results in vessel wall thickening, mural edema, and mural enhancement. Following this phase, progressive narrowing of the vessel occurs which results in vascular occlusion or stenosis. This phase is also known as pulseless/occlusive phase.[1],[2] The patient usually presents with signs and symptoms of ischemia during this phase, such as arm claudication, pulse deficit, and abdominal angina. Stenosis is most commonly seen in the aorta, and it mostly involves descending aorta. While in case of abdominal aorta, renal artery is most frequently involved. Occlusion of the vessels may lead to the development of aneurysm or collateral circulation.[3],[4] Diagnosis of TA is mainly based on the clinical evaluation, laboratory parameters, and imaging modalities. TA is primarily involves large vessels but may also cause medium-vessel vasculitis. CTA is most specific for the diagnosis of TA. Concentric thickening of the vessel wall and its calcification are the most important findings on CTA and best evaluated on the axial images of CTA. The thickened wall gives the appearance of “double ring” in which there are a poorly enhanced internal ring and a well-enhanced outer ring.[4],[5] This sign is best appreciated in the venous phase. The mural thickness may vary up to several millimeters. In our case, the thickness of the vessel wall was around 6.0 mm which is an indicative of active disease.[3] Calcification if present is mainly transmural. The most common finding associated with mural thickening is stenosis, which is an important finding in our case. Other than stenosis, occlusion, aneurysm, and ectasia of the vessels can also be seen. Dilatation is usually seen in the ascending aorta, while aneurysms are commonly seen in the abdominal aorta. These conditions can lead to aortic rupture which is life-threatening. Sometimes, tapered distal segment of the involved vessel with the normal or dilated lumen of the proximal segment can give a characteristic “rat tail”-like appearance.[4] In our case, CTA revealed significant narrowing of the descending aorta with stenosis of the left renal artery (Type III of angiographic classification of TA).[4] Usually, renal infarct occurs due to medium-vessel vasculitis. Therefore, in our case, along with stenosis of the left renal artery (large-vessel vasculitis), there is a high possibility of medium-vessel vasculitis causing an infarct in the lower pole of the left kidney which is a rare complication of TA. This suggests that any type of vessel can be involved in any form of vasculitis.[6]
Figure 1: Computed tomography angiography axial section (a) and coronal reformat (b) showing significant smooth narrowing of the left renal artery (white arrow) and left renal infarct in the lower pole (yellow star)

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Figure 2: Reformatted computed tomography angiography coronal (a) and sagittal (b) sections showing significant smooth long segment narrowing of descending aorta (white arrow) and left renal artery (yellow arrow)

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Treatment of TA is mainly medical which involves life-long consumption of steroids. If a patient presents with complications such as stenosis or occlusion of vessels, angioplasty, stenting, or bypass surgery can be done. In cases of comorbid conditions such as hypertension in our case, early identification and aggressive treatment of the same may alleviate the symptoms.[5]

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient's parents have given their consent for her images and other clinical information to be reported in the journal. The patient's parents understand that her names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Ricardo RA, Katsicas MM. Takayasu arteritis. Front Pediatr 2018;6:265.  Back to cited text no. 1
    
2.
Gao S, Wang R. Takayasu arteritis presenting with massive cerebral ischemic infarction in a 35-year-old woman: A case report. J Med Case Rep 2013;7:179.  Back to cited text no. 2
    
3.
Gaballah M, Goldfisher R, Amodio JB. The utility of MRI in the diagnosis of Takayasu arteritis. Case Rep Pediatr 2017;2017:7976165.  Back to cited text no. 3
    
4.
Zhu FP, Luo S, Wang ZJ, Jin ZY, Zhang LJ, Lu GM. Takayasu arteritis: Imaging spectrum at multidetector CT angiography. Br J Radiol 2012;85:e1282-92.  Back to cited text no. 4
    
5.
Ethiraj D, Kanase ND, Indiran V. “Double ring sign in Takayasu arteritis.” QJM 2020:hcaa030. [Doi: doi.org/10.1093/qjmed/hcaa030].  Back to cited text no. 5
    
6.
Misra DP, Jain N, Harikrishnan G, Agarwal V. Multiple jeopardy: Diagnostic and therapeutic challenges in vasculitic flare. Int J Rheum Dis 2020;23:697-701.  Back to cited text no. 6
    


    Figures

  [Figure 1], [Figure 2]



 

 
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