Saudi Journal of Kidney Diseases and Transplantation

: 2008  |  Volume : 19  |  Issue : 4  |  Page : 643--645

Calcific Uremic Arteriolopathy in a Patient on Hemodialysis

Faissal Tarrass, Meryem Benjelloun 
 Department of Nephrology and Dialysis, Hassani General Hospital, 62000 Nador, Morocco

Correspondence Address:
Faissal Tarrass
Department of Nephrology and Dialysis, Hassani General Hospital, 62000 Nador

How to cite this article:
Tarrass F, Benjelloun M. Calcific Uremic Arteriolopathy in a Patient on Hemodialysis.Saudi J Kidney Dis Transpl 2008;19:643-645

How to cite this URL:
Tarrass F, Benjelloun M. Calcific Uremic Arteriolopathy in a Patient on Hemodialysis. Saudi J Kidney Dis Transpl [serial online] 2008 [cited 2021 Feb 27 ];19:643-645
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Full Text

To the Editor,

A 53-year-old man with end-stage renal disease (ESRD) due to autosomal dominant polycystic kidney disease started treatment with hemodialysis in 2001. He had hyper­tension and did not have any other co­morbidities. In 2007, the patient developed uncontrollable secondary hyperparathyroi­dism. Activated vitamin D was relatively contra-indicated due to a persistently ele­vated serum calcium phosphate product (4.90-7.90 mmol 2 /l 2 ). Pre-dialysis corrected serum calcium was at or above the upper limit of normal range at 2.60-2.72 mmol/l and serum phosphate was elevated between 1.67-2.95 mmol/l, despite therapy with seve­lamer. Intact parathyroid hormone levels had reached 1080 pg/ml. The patient developed pain in his fingers, and in September 2007 he developed rapidly progressive painful ery­thematous plaques, followed by ulceration of his 3rd, 4th and 5th left fingers. Digital ulceration expanded in the following days, rapidly evolving into total digital gangrene [Figure 1]. The patient underwent disarti­culation of the left 3rd and 4th fingers with debridement of necrotic tissues, because there was no possibility of revascularization. Microscopic examination showed calcifica­tion of the media and occlusive hyperplasia of the intima of small-to-medium sized arte­ries, suggestive of calcific uremic arterio­lopathy (CUA). Emergency subtotal para­thyroidectomy was then performed, and the pathology revealed encapsular nodular hy­perplasia of the parathyroid glands. After surgery, the patient had no pain, and made good recovery without new lesions.

CUA was first described as calciphylaxis by Hans Selye in 1962; [1] the term calcific uremic arteriolopathy is now preferred. [2] CUA is a clinical syndrome characterized by painful and pruritic skin lesions, subcu­taneous nodules, skin necrosis, ulceration, and eschar formation, observed mainly in patients with ESRD on renal replacement therapy or after renal transplantation. [3],[4],[5] This syndrome occurs in 1-4% of patients on long-term hemodialysis (HD), and is asso­ciated with high morbidity and mortality re­sulting primarily from local and systemic infections. [6] Histopathological examination of biopsy material from such patients typically reveals a generalized involvement of small arteries in numerous organs with medial calcification and intimal proliferation with microthrombi. [7]

CUA has been reported in patients with severe hyperparathyroidism; [8] however, a few cases have been described having low le­vels of parathyroid hormone and some had even undergone parathyroidectomy. [9],[10] The affected areas are usually the toes, thighs,and lower abdomen and even the breast. [3] However, digital gangrene as primum mo­vens of CUA has not been reported earlier in the nephrology literature.

A case-control study demonstrated that raised serum phosphate concentrations were associated with a substantially increased risk of CUA and that calcium-phosphate product values tended to be higher in affected patients than in controls. [4] Emerging evidence suggests that the process of vas­cular calcification may be more complicated than simple mineral precipitation. Various proteins involved in the control of bone and mineral metabolism are expressed in calcified arterial lesions. [11] This would sug­gest that vascular calcification is not sim­ply a passive process related to serum calcium and phosphate homeostasis but is an actively mediated one.

There are no randomized controlled trials to guide management of patients with CUA. A number of strategies may be used in combination. [12],[13] Wound infections must be treated promptly and aggressively. [13],[14] Serum calcium should be reduced by using a low­calcium dialysate and by discontinuing calcium containing phosphate binders. Serum phosphate levels are reduced by increasing the dialysis dose and using non calcium containing phosphate binders. [7],[8],[10],[12] There is some evidence to suggest that hyper­baric oxygen therapy may improve outcome. [6]

In patients with severe secondary or tertiary hyperparathyroidism, parathyroidec­tomy should be considered. [8],[12] Calcimimetic agents also provide a novel therapeutic ap­proach for controlling secondary HPT. [13],[15]

In conclusion, this is the first report, to our knowledge, of CUA presented as digi­tal gangrene. Physicians should be aware of this feature of CUA in patients with ESRD on hemodialysis.


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