| |
|
|
| Year : 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
Click here for correspondence address and email
|
|
 |
|
How to cite this article:
Tarrass F, Benjelloun M. Calcific Uremic Arteriolopathy in a Patient on Hemodialysis. Saudi J Kidney Dis Transpl 2008;19:643-5 |
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 2020 Nov 26];19:643-5. Available from: https://www.sjkdt.org/text.asp?2008/19/4/643/41329 |
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.
 References | |  |
| 1. | Selye H. Calciphylaxis. Chicago: University of Chicago Press. 1962:1-16.  |
| 2. | Finucane KA, Archer CB. Dermatological aspects of medicine: recent advances in nephrology. Clin Exp Dermatol 2005;30 (1):98-102. |
| 3. | Hafner J, Keusch G, Wahl C, et al. Uremic small artery disease with medial calcification and intimal hyperplasia (so called calciphylaxis: a complication of chronic renal failure and benefit from parathyroidectomy. J Am Acad Dermatol 1995;33 (6):954-62. |
| 4. | Mazhar AR, Johnson RJ, Gillen D, et al. Risk factors and mortality associated with calciphylaxis in end-stage renal disease. Kidney Int 2001;60(1):324-32. |
| 5. | Brewster UC, Perazella MA. Calcific uremic arteriolopathy in a transplanted kidney. Am J Med Sci 2005;329(2):102-3. |
| 6. | Podymow T, Wherrett C, Burns KD. Hyperbaric oxygen in the treatment of calciphylaxis: a case series. Nephrol Dial Transplant 2001;16(11):2176-80. |
| 7. | Coates T, Kirkland GS, Dymock RB, et al. Cutaneous necrosis from calcific uremic arteriolopathy. Am J Kidney Dis 1998;32 (3):384-91. |
| 8. | Duh QY, Lim RC, Clark OH. Calciphylaxis in secondary hyperparathyroidism: diagnosis and parathyroidectomy. Arch Surg 1991;126(10):1213-9. |
| 9. | Pliquett RU, Schwock J, Paschke R, Achenbach H. Calciphylaxis in chronic, non-dialysis-dependent renal disease. BMC Nephrol 2003;4:8. [PUBMED] [FULLTEXT] |
| 10. | Oikawa S, Osajima A, Tamura M, et al. Development of proximal calciphylaxis with penile involvement after parathyroidectomy in a patient on hemodialysis.Int Med 2004;43(1):63-8. |
| 11. | Ahmed S, O'Neill KD, Hood AF, Evan AP, Moe SM. Calciphylaxis is associated with hyper-phosphatemia and increased osteopontin expression by vascular smooth muscle cells. Am J Kidney Dis 2001;37 (6):1267-76. |
| 12. | Don BR, Chin AI. A strategy for the treatment of calcific uremic arteriolopathy (calciphylaxis) employing a combination of therapies. Clin Nephrol 2003;59(6):463-70. |
| 13. | Sharma A, Burkitt-Wright E, Rustom R. Cinacalcet as an adjunct in the successful treatment of calciphylaxis. Br J Dermatol 2006;155(6):1295-7. |
| 14. | Tittelbach J, Graefe T, Wollina U. Painful ulcers in calciphylaxis-combined treatment with maggot therapy and oral pentoxyfillin. J Dermatol Treat 2001;12(4):211-4. |
| 15. | Robinson MR, Augustine JJ, Korman NJ. Cinacalcet for the treatment of calciphylaxis. Arch Dermatol 2007;143(2):152-4. |
Correspondence Address:
Faissal Tarrass
Department of Nephrology and Dialysis, Hassani General Hospital, 62000 Nador
Morocco
  | Check |
PMID: 18580028 
[Figure 1] |
|
|
|
|
|
|
|
|
|
|
|
|
|
| Article Access Statistics | | | Viewed | 3196 | | | Printed | 95 | | | Emailed | 0 | | | PDF Downloaded | 377 | | | Comments | [Add] | | |
|
|
