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   Abstract
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   Discussion
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CASE REPORT
Year : 2007  |  Volume : 17  |  Issue : 2  |  Page : 78-79
 

Extensive calcific uremic arteriolopathy in a patient on automated peritoneal dialysis


1 Department of Nephrology, Ram Manohar Lohia Hospital, New Delhi, India
2 Department of Medicine (Division of Nephrology), University College of Medical Sciences, University of Delhi, New Delhi, India

Correspondence Address:
O P Kalra
Division of Nephrology, University College of Medical Sciences and G T B Hospital, Dilshad Garden, New Delhi
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0971-4065.37028

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  Abstract 

Calciphylaxis, better known as calcific uremic arteriolopathy at present, is a relatively uncommon condition characterized by small vessel calcification and occlusion with resultant painful violaceous skin lesions that typically ulcerate to form nonhealing gangrenous ulcers. The mortality of the disease is very high mainly due to sepsis from superinfection. This syndrome is usually observed in diabetic patients with low bone turnover having low parathyroid hormone. Use of noncalcium containing phosphate binders along with the care of the affected part is helpful in the overall recovery.


Keywords: Aortic calcification, calcification, calciphylaxis, coronary artery, cutaneous ulcers, soft tissue calcification, vascular calcification


How to cite this article:
Sunder S, Kalra O P, Waghmare V, Ruchi R, Raizada A. Extensive calcific uremic arteriolopathy in a patient on automated peritoneal dialysis. Indian J Nephrol 2007;17:78-9

How to cite this URL:
Sunder S, Kalra O P, Waghmare V, Ruchi R, Raizada A. Extensive calcific uremic arteriolopathy in a patient on automated peritoneal dialysis. Indian J Nephrol [serial online] 2007 [cited 2019 Nov 22];17:78-9. Available from: http://www.indianjnephrol.org/text.asp?2007/17/2/78/37028



  Introduction Top


Vascular calcification (VC) is the deposition of calcium-phosphate complexes in the form of bioapatite [1] in the myocardium, cardiac valves and the blood vessels. It is responsible for high cardiovascular mortality in end stage renal disease (ESRD) and may result in calciphylaxis - also known as calcific uremic arteriolopathy (CUA) - a necrotizing skin condition associated with high mortality rates. [2] In this study, we report a case of ESRD on automated peritoneal dialysis (APD), who developed diffuse CUA associated with gluteal ulceration and painful nodular lesions over the lower limbs and discuss various pathogenetic mechanisms and the current treatment options for this serious condition.


  Case Report Top


A 65-year-old female had been a known case of diabetes mellitus for 20 years and hypertension and coronary artery disease for the past 10 years. Five years back, her disease progressed to ESRD and she was started on APD. She gave a history of left renal calculus in the past, which was removed 15 years back. Initial investigations performed 5 years back revealed the following: hemoglobin - 8.6 g/dl, normal total and differential cell counts, blood urea - 156 mg/dl, serum creatinine - 8.6 mg/dl, serum calcium - 8.7 mg/dl, serum phosphorus - 4.6 mg/dl (Ca x P: 40.02), alkaline phosphatase - 300 IU/l and intact PTH level (iPTH) - 386 pg/ml. She was put on treatment with iron, calcium containing phosphate binders, vitamin D 3 , low-dose aspirin, antihypertensive and antianginal medication. Repeat investigations done 3 years back revealed the following: serum calcium - 9.0 mg/dl, serum phosphorus - 5.6 mg/dl (Ca x P: 50.4). Her serum alkaline phosphatase was 150 IU/L and iPTH level was 299 pg/ml.

One year back, she presented with complaints of backache, joint pains, right gluteal ulcer and painful nodules on both the lower limbs. Her general physical examination revealed multiple, tender 1- mm-sized nodules on both the lower limbs and an ulcer on the medial aspect of right gluteal region, with no signs of inflammation. Her blood pressure was 150/96 mmHg, and she had no cyanosis, clubbing or pedal edema. All the peripheral pulses were well palpable. Her peripheral joints were normal with no evidence of arthritis and her systemic examination was within the normal limits. Her investigations revealed the following: hemoglobin - 9 g/dl, ESR - 54 mm at the end of the first hour, normal total and differential cell counts, blood urea - 170 mg/dl, serum creatinine - 9.0 mg/dl, serum albumin - 3.1 g/dl and her coagulation profile was normal. She had a serum calcium level of 9.8 mg/dl and, serum phosphorus level of 9.2 mg/dl (Ca x P: 90.16). Her serum alkaline phosphatase level was 100 IU/L and iPTH level was 90 pg/ml, which was suggestive of a low turnover disease.

Her hepatitis B surface antigen and anti-HCV serology were negative. Her ECG showed left ventricular hypertrophy with strain pattern and two-dimensional echocardiography revealed moderate mitral regurgitation, aortic regurgitation, calcific atherosclerosis with a diastolic dysfunction and LVEF of 45%. Her radiological survey revealed the calcification of the trachea, aorta, radial and femoral arteries and soft tissue calcification. Her plain cardiac CT scan revealed coronary artery calcium score of 3437 (AJ - 130). A coronary artery calcium score higher than 400 is indicative of extensive plaque burden and even coronary artery calcification. She was advised to discontinue calcium-containing phosphate binders and vitamin D and was put on treatment with oral sevelamer. Besides this, debridement and local care of the gluteal ulcer was performed. Consequently, her serum calcium and phosphate levels returned to normal, gluteal ulcer healed and there was marked improvement of her symptoms over the next few months.


  Discussion Top


During the initial few years, our patient had evidence of secondary hyperparathyroidism; however, she subsequently developed features of adynamic bone disease characterized by low iPTH levels and raised calcium-phosphorus product resulting in CUA. It may be observed in renal failure patients before and after treatment with peritoneal dialysis or hemodialysis. Its prevalence varies between 15% and 60% among the dialysis patients and 30% in predialysis patients and is more common in patients with diabetes mellitus. It is characterized by a defect in the bone matrix formation and mineralization, increased osteoid thickness and a decrease in the number of both osteoblasts and osteoclasts on the bone surface. Calcium uptake by the adynamic bone is reduced and therefore some patients may develop hypercalcemia if the calcium intake is increased or if the dialysate calcium is high and may result in calcification in the blood vessels and other tissues.

Vascular calcification is an actively regulated process that may arise by various mechanisms such as loss of inhibition of VC, [3],[4] induction of bone formation, [5] circulating nucleational complexes. [6],[7] Besides this, the raised calcium-phosphate product may lead to apatite nucleation and crystal growth by passive mechanisms. [1] Furthermore, new evidence suggests that calcium and phosphorus may additionally have direct effects on the vascular cells that predispose to mineralization. [8],[9] It has been shown that the blood vessels normally express the inhibitors of mineralization, such as pyrophosphate and matrix GLA protein and the loss of these molecules may predispose to vascular calcification. [3],[4] Other factors include phenotypic changes in the vascular media with cells leading to induction of osteogenic mechanisms [5] and the release of circulating nucleational complexes due to bone turnover.

Calcific uremic arteriolopathy is a potentially life-threatening disease that occurs in upto 4% of patients with ESRD, being fatal in 60-80% of patients. [2] It presents as subcutaneous lesions with livedo reticularis and painful violaceous skin lesions leading to ulceration predominantly in the lower limbs and the abdomen due to small vessel calcification and occlusion. [10] Treatment options for VC include the use of noncalcium-containing phosphate binders such as sevelamer, lanthanum carbonate and parathyroidectomy. A recent study has shown the benefit of the calcium channel blocker, nifedipine and endothelin-1 receptor antagonist in the preventing and slowing of VC. [1]

 
  References Top

1.Giachelli CM. Vascular calcification mechanisms. J Am Soc Nephrol 2004;15:2959-64.  Back to cited text no. 1  [PUBMED]  [FULLTEXT]
2.Trost O, Kadlub N, Trouilloud P, Malka G, Danino A. Calciphylaxis: A severe but unrecognized complication in end-stage renal disease patients: A review of 2 cases. Ann Chir Plast Esthet 2005;50:746-50.  Back to cited text no. 2    
3.Rutsch F, Ruf N, Vaingankar S, Toliat MR, Suk A, Hohne W, et al. Mutations in ENPP1 are associated with "idiopathic" infantile arterial calcification. Nat Genet 2003;34:379-81.  Back to cited text no. 3    
4.Luo G, Ducy P, McKee MD, Pinero GJ, Loyer E, Behringer RR, et al. Spontaneous calcification of arteries and cartilage in mice lacking matrix GLA protein. Nature 1997;386:78-81.  Back to cited text no. 4  [PUBMED]  [FULLTEXT]
5.Tintut Y, Parhami F, Bostrom K, Jackson SM, Demer LL. cAMP stimulates osteoblast-like differentiation of calcifying vascular cells: Potential signaling pathway for vascular calcification. J Biol Chem 1998;273:7547-53.  Back to cited text no. 5    
6.Price PA, Caputo JM, Williamson MK. Bone origin of the serum complex of calcium, phosphate, fetuin, and matrix Gla protein: Biochemical evidence for the cancellous bone-remodeling compartment. J Bone Miner Res 2002;17:1171-9.  Back to cited text no. 6    
7.Price PA, Faus SA, Williamson MK. Bisphosphonates alendronate and ibandronate inhibit artery calcification at doses comparable to those that inhibit bone resorption. Arterioscler Thromb Vasc Biol 2001;21:817-24.  Back to cited text no. 7  [PUBMED]  [FULLTEXT]
8.Jono S, McKee MD, Murry CE, Shioi A, Nishizawa Y, Mori K, et al. Phosphate regulation of vascular smooth muscle cell calcification. Circ Res 2000;87:E10-7.  Back to cited text no. 8  [PUBMED]  [FULLTEXT]
9.Chen NX, O'Neill KD, Duan D, Moe SM. Phosphorus and uremic serum up-regulate osteopontin expression in vascular smooth muscle cells. Kidney Int 2002;62:1724-31.  Back to cited text no. 9  [PUBMED]  [FULLTEXT]
10.Lang N, Davie R, Whitworth C, Winney R, Hughes J. Fatal calcific uraemic arteriolopathy (CUA): A case report and review of literature. Scott Med J 2004;49:108-11.  Back to cited text no. 10  [PUBMED]  




 

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Indian Journal of Nephrology
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