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Saudi Journal of Kidney Diseases and Transplantation
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Table of Contents   
CASE REPORT  
Year : 2015  |  Volume : 26  |  Issue : 5  |  Page : 966-969
An unusual case of a patient who lost his native kidneys and renal allograft from cholesterol crystal emboli


1 Department of Medicine, King Abdul Aziz Hospital, National Guard Health Affairs, King Abdullah International Medical Research Center/King Saud bin Abdulaziz University for Health Sciences, Al Ahsa, Saudi Arabia
2 Department of Pathology, King Abdul Aziz Hospital, National Guard Health Affairs, King Abdullah International Medical Research Center/King Saud bin Abdulaziz University for Health Sciences, Al Ahsa, Saudi Arabia

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Date of Web Publication7-Sep-2015
 

   Abstract 

Cholesterol crystal emboli (CCE) syndrome involving native kidneys is an underdiagnosed condition. CCE is rare in renal allografts. It may present with acute kidney injury, but usually not acute graft loss. CCE should be considered in patients with a history of atherosclerosis and an invasive arterial procedure who present with acute or chronic renal allograft dysfunction. Therapy for CCE is mainly supportive and carries a high rate of mortality. To the best of our knowledge, this is the first reported case of a patient who lost his native kidneys and renal allograft due to CCE arising from his own vasculature.

How to cite this article:
Ahmed W, Al Garni A, Abdelgadir E, Khamees KO, Ellouly MA, Haleem A. An unusual case of a patient who lost his native kidneys and renal allograft from cholesterol crystal emboli. Saudi J Kidney Dis Transpl 2015;26:966-9

How to cite this URL:
Ahmed W, Al Garni A, Abdelgadir E, Khamees KO, Ellouly MA, Haleem A. An unusual case of a patient who lost his native kidneys and renal allograft from cholesterol crystal emboli. Saudi J Kidney Dis Transpl [serial online] 2015 [cited 2020 Aug 3];26:966-9. Available from: http://www.sjkdt.org/text.asp?2015/26/5/966/164580

   Introduction Top


Cholesterol crystal emboli (CCE) is a systemic disease resulting from cholesterol crystal embolization and leading to the occlusion of small vessels in a variety of organs, including the kidneys, skin, brain, gastrointestinal tract and extremities. [1] CCE occurs in patients with diffuse erosive atherosclerosis, usually triggered by aortic surgery, arterial invasive procedures and also after anticoagulant and thrombolytic therapy. [2] The proximity of the kidneys to the abdominal aorta and the large renal blood vessels make the kidney a target organ for CCE.


   Case Report Top


A 59-year-old Saudi male patient presented to the hospital with backache. He had a past history of live-related renal transplant in 2008, diabetes mellitus, hypertension, dyslipidemia an abdominal aortic aneurysm (AAA) and coronary artery disease. The allograft was placed in the right iliac fossa and anastomosed to the right external iliac vessels. His baseline serum creatinine was 109 μmol/L.

An abdominal computed tomography (CT) scan showed a 5-cm diameter infra-renal AAAwithout evidence of leak or rupture but being at high risk of rupture. His serum creatinine at this time was 93 μmol/L. Other laboratory investigations were unremarkable.

The patient preferred to go to another center for the endovascular procedure. On 24 th December 2011, he underwent stenting of the abdominal aneurysm, open arteriotomy to both femoral arteries and balloon dilation of the right external iliac artery. At the time of discharge, his creatinine level was 118 μmol/L, which later improved to 106 μmol/L.

Fifteen days after his procedure, he presented to our center with generalized fatigability and loss of appetite. On examination, he looked slightly dehydrated and other systemic examination was unremarkable.

Investigations at this time showed elevation of serum creatinine to 159 μmol/L. His complete blood count was unremarkable, with eosinophils 1%, C-reactive protein was 23 mg/L and normal complements levels. Serum tacrolimus level (T 0 ) was 5.3 ng/mL. Urinalysis was unremarkable. Urine, blood cultures, Cytomegalovirus serology and urine eosinophils were all negative. Chest X-ray, ultrasound and Doppler of the renal allograft was by transthoracic and transesophageal echocardiography.

The initial impression was volume depletion causing rise in serum creatinine. The patient was started on intravenous fluids and serum creatinine improved to 119 μmol/L over the next four days. However, after that, the serum creatinine rose again rapidly and reached 306 μmol/L within a few days.

As an immediate biopsy could not be performed because of the weekend, he was pulsed with three doses of 1 g of intravenous methylprednisolone daily and mycophenolate mofetil was increased to 1 g twice daily to cover for a possible acute rejection.

Allograft biopsy was performed. Biopsy showed background chronic transplant nephropathy with patches of tubular atrophy and focal infiltrate of mononuclear cells. There was no evidence of tubulitis or any neutrophilic stuffing in the peritubular capillaries. However, it showed the arteries to be stuffed with cholesterol emboli [Figure 1] and [Figure 2] . He did not have any other signs of CCE. His renal function continued to deteriorate rapidly and he became dialysis dependant within a few days.
Figure 1: Transplant renal biopsy showing two arteries, one each in the opposite angles of this picture with cholesterol crystals as indicated by clear spaces. The renal parenchyma shows patches of tubular atrophy and mononuclear cell infiltrate.

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Figure 2: High magnification of one of the arteries in Figure 1. The arterial lumen is occluded by cholesterol crystals with associated inflammatory reactions.

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Interestingly, he had developed end-stage renal failure in 2005 due to CCE of his native kidneys. This was approximately two weeks post-cardiac catheterization. He had clinical features of CCE in the form of abdominal pain, bluish discoloration of his toes and eosinophilia (7%). He was started on hemodialysis until he received his renal transplant in 2008. To the best of our knowledge, this is the first reported case of a patient losing his native and renal allograft due to CCE arising from his own vasculature.


   Discussion Top


The Danish physician Fenger and his colleagues provided the first description of atheroembolism in the Danish medical brochure Ugeskrift for Laeger (Doctors'Weekly). [3] The first autopsy series was reported in 1945 from the New York Hospital by Flory. [4] The first ante-mortem non-invasive diagnosis of cholesterol embolization syndrome was described in 1961 by Dr. Robert Hollenhorst (1913-2008), an ophthalmologist working at the Mayo Clinic. [5]

Cholesterol embolization in native kidneys is uncommon, having an incidence of approximately 1.1-2.7% in native renal biopsies. [6],[7],[8]

The symptoms of CCE can be non-specific, which include leg, toe or foot pain, fever, myalgias, weight loss and abdominal, flank or back pain. Signs include blue toes syndrome, livedo reticularis, gangrene, gross hematuria, accelerated hypertension and renal failure. Neurological features of CCE include headache, amaurosis fugax, stroke, altered mental status, paraparesis and mononeuropathy. [2]

CCE is a rare occurrence in renal allograft with a reported incidence of 0.47% in one study. [9] Graft failure is significantly associated with donor-derived and early CCE. [10] Eosinophilia may be absent in 20% of the patients. [11] Hypocomplementemia is seen only in 30-40% of patients. Histological diagnosis can be achieved by skin and renal biopsy. [2]

Treatment consists of supportive care and secondary prophylaxis against further episodes of CCE. [12] There is no evidence that antiinflammatory treatments are beneficial in CCE. There is also no direct evidence that antiplatelet agents and angiotensin converting enzyme inhibitors directly prevent the recurrence of CCE syndrome. [13]

There is some evidence that statin therapy decreases the risk of cholesterol embolization syndrome. Unfortunately, there has been no randomized trial of statin therapy in patients with severe aortic plaque. [11]

Peripheral eosinophilia suggests a possible role for inflammation in the pathogenesis of renal CCE, which supports the rationale to use corticosteroids in this disease. [8] Steroid therapy showed a good renal outcome in CCE patients. However, this treatment did not have a favorable effect on long-term renal outcome. [12]

The use of thrombolytic therapy and anticoagulants in CCE remains controversial. About 30-55% patients with renal involvement will need renal replacement therapy. Mortality is high, with 15-30% of patients dying during the first year. [13],[14]


   Conclusion Top


It is important to know the patient's past medical history in clinical decision making and patient management. Risks and benefits of any invasive procedure must be weighed before being performed, should be explained to the patient and should be documented in the written consent.

As donors and recipients of the elderly age group or with prominent atherosclerosis are accepted for transplantation, CCE renal disease may become even more prevalent in the future. Patients may present with non-specific symptoms. CCE should be considered in patients with a history of atherosclerosis and an invasive arterial procedure who present with acute or chronic renal allograft dysfunction.

Conflict of interest: None

 
   References Top

1.
Scoble JE, O'Donnell PJ. Renal atheroembolic disease: The Cinderella of nephrology? Nephrol Dial Transplant 1996;11:1516-7.  Back to cited text no. 1
    
2.
Granata A, Insalaco M, Di Pietro F, Di Rosa S, Romano G, Scuderi R. Atheroembolism renal disease: Diagnosis and etiologic factors. Clin Ter 2012;163:313-22.  Back to cited text no. 2
    
3.
Fenger CE, Jacobsen JP, Dahlerup EA, Hornemann E, Collin T. Beretning af obductionen over Albert Thorvaldsen (autopsy report of Albert Thorvaldsen). Ugeskr Laeger 1844;X:215-8.  Back to cited text no. 3
    
4.
Flory CM. Arterial occlusions produced by emboli from eroded aortic atheromatous plaques. Am J Pathol 1945;21:549-65.  Back to cited text no. 4
    
5.
Hollenhorst RW. Significance of bright plaques in the retinal arterioles. JAMA 1961;178:23-9.  Back to cited text no. 5
    
6.
Greenberg A, Bastacky SI, Iqbal A, Borochovitz D, Johnson JP. Focal segmental glomerulosclerosis associated with nephrotic syndrome in cholesterol atheroembolism: Clinicopathological correlations. Am J Kidney Dis 1997;29:334-44.  Back to cited text no. 6
    
7.
Jones DB, Iannaccone PM. Atheromatous emboli in renal biopsies. An ultrastructural study. Am J Pathol 1975;78:261-76.  Back to cited text no. 7
    
8.
Modi KS, Rao VK. Atheroembolic renal disease. J Am Soc Nephrol 2001;12:1781-7.  Back to cited text no. 8
    
9.
Ripple MG, Charney D, Nadasdy T. Cholesterol embolization in renal allografts. Transplantation 2000;69:2221-5.  Back to cited text no. 9
    
10.
Lai CK, Randhawa PS. Cholesterol embolization in renal allografts: A clinicopathologic study of 12 cases. Am J Surg Pathol 2007;31:536-45.  Back to cited text no. 10
    
11.
Nakayama M, Izumaru K, Nagata M, et al. The effect of low-dose corticosteroids on shortand long-term renal outcome in patients with cholesterol crystal embolism. Ren Fail 2011;33:298-306.  Back to cited text no. 11
    
12.
Kronzon I, Tunick PA. Aortic atherosclerotic disease and stroke. Circulation 2006;114:63-75.  Back to cited text no. 12
    
13.
Belenfant X, Meyrier A, Jacquot C. Supportive treatment improves survival in multivisceral cholesterol crystal embolism. Am J Kidney Dis 1999;33:840-50.  Back to cited text no. 13
    
14.
Frank RD. Cholesterol embolism syndrome: A rare, but severe complication in patients with atherosclerosis. Dtsch Med Wochenschr 2012;137:1130-4.  Back to cited text no. 14
    

Top
Correspondence Address:
Wasim Ahmed
Department of Medicine (MC 312), King Abdul Aziz Hospital, National Guard Health Affairs, P. O. Box 2477, Post Code 31982, Al Ahsa
Saudi Arabia
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DOI: 10.4103/1319-2442.164580

PMID: 26354570

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    Abstract
   Introduction
   Case Report
   Discussion
   Conclusion
    References
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