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Saudi Journal of Kidney Diseases and Transplantation
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Year : 2010  |  Volume : 21  |  Issue : 2  |  Page : 323-327
Catastrophic calciphylaxis in a patient with lupus nephritis and recent onset of end-stage renal disease


Department of Internal Medicine, Nephrology Division, King Abdulaziz University Hospital, Jeddah, Saudi Arabia

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Date of Web Publication9-Mar-2010
 

   Abstract 

Painful violaceous skin lesions that progress to non-healing ulceration and gangrene characterize calciphylaxis. These lesions are associated with secondary hyperparathyroidism and generally occur in patients on dialysis for more than one year. Hyperphosphatemia and hypoalbumi­nemia are the major risk factors for calciphylaxis. It is usually resistant to medical treatment al­though parathyroidectomy can help in controlling the disease. The mortality rate of calciphylaxis is very high due to uncontrollable sepsis. In our case, a young female with systemic lupus erythema­tosus (SLE) developed calciphylaxis within a short period after the onset of hemodialysis; she had a short period of hyperphosphatemia prior to dialysis. The serum phosphate was 4.24 mmol/L, cal­cium was 1.66 mmol/L, parathormone was 38 and calcium-phosphate was 7.0 mmol/L. It is likely that SLE provoked the development of calciphylaxis. The patient was treated medically but un­fortunately died secondary to sepsis.

How to cite this article:
Al Beladi FI. Catastrophic calciphylaxis in a patient with lupus nephritis and recent onset of end-stage renal disease. Saudi J Kidney Dis Transpl 2010;21:323-7

How to cite this URL:
Al Beladi FI. Catastrophic calciphylaxis in a patient with lupus nephritis and recent onset of end-stage renal disease. Saudi J Kidney Dis Transpl [serial online] 2010 [cited 2020 Dec 4];21:323-7. Available from: https://www.sjkdt.org/text.asp?2010/21/2/323/60204

   Introduction Top


Calciphylaxis is a pathophysiological change that is caused by the deposition of calcium which leads to painful skin lesions and often cutaneous necrosis. [1] Calciphylaxis is an uncom­mon condition that occurs in patients with chronic kidney failure in association with se­condary hyperparathyroidism. When kidney func­tion is normal, calciphylaxis may be associated with breast cancer, [2],[3] primary hyperparathyroi­dism, [4],[5] short bowel syndrome, Crohn's disease, [6] protein S deficiency, rheumatoid arthritis, [7] and acute renal failure in alcoholic cirrhosis. [8]

Several risk factors for calciphylaxis have been identified. These include obesity, diabetes, Cau­casian race, female gender, hypoalbuminemia, and a calcium-phosphate product (a-p) of 70 or more. [9] Clinical history and physical examina­tion can be sufficient to diagnose calciphylxis. A biopsy of the ulcerated wound is the most accurate method to confirm the diagnosis. His­tological examination shows medial calcifica­tion, and intimal fibrous hyperplasia of arterioles, capillaries, and venules.

Calciphylaxis is associated with high morbi­dity and mortality. To prevent calciphylaxis, aggressive management of calcium, phosphate, and parathyroid hormone levels are critical. If skin lesions occur, oral corticosteriod is effec­tive in addition to aggressive local surgical care, and parathyriodectomy. In this paper, a case of calciphylaxis secondary to systemic lupus ery­thematosus (SLE) is reported.


   Case History Top


In late 2004, a 23-year-old female patient, who presented with polyarthralgia, mouth ulcers, and alopecia, was diagnosed to have SLE compli­cated by class IV nephritis, in another hospital. Antinuclear antibody (ANA) and double stranded DNA antibody (dsANA) were positive. At that time, renal function was normal. Liver function was normal except low serum albumin, 12 gm/L (NR 34-40). She was treated with methyl­prednisolone (1g i.v.) for three days followed by oral prednisolone (60 mg) in a tapering dose. The total duration of treatment was not very clear. Since 2004, the patient had frequent re­lapses of disease activity and each time she was treated with pulse steroids followed by a tape­ring dose of prednisolone.

In March 2006, the woman was admitted to the King Abdulaziz University Hospital (KAUH), Jeddah, Saudi Arabia with dyspnea, lower-limb edema, decreased urine output and polyarthral­gia. On clinical examination, the following fin­dings were noted: body temperature, 37.1°C; blood pressure, 149/90 mmHg; heart rate, 84 beats/min; elevated jugular venous pressure (JV­P); body weight, 90 kg and lower limb edema. Chest examination revealed bilateral basal cre­pitations. The remainder of the examination was unremarkable. Laboratory tests revealed high creatinine level of 402 mmol/L (NR 53-115), active urine sediment, high phosphate of 3.3 mmol/L (NR 0.81-1.58), calcium of 2.42 mmol/L (2.12-2.52), albumin of 19 gm/L and alkaline phosphatase of 63 IU/L (NR 53-115). Repeat renal biopsy showed diffuse proliferative glo­merulonephritis with crescents, consistent with class IV lupus nephritis. She was treated with pulse methylprednisolone (1 gm i.v.) for three days followed by oral prednisolone (60 mg dai­ly) for one month, to be slowly tapered. At the same time, treatment was started with myco­phenolate mofetil, 1 gm twice daily. The patient responded to hemodialysis and began to pro­duce urine in the range of 1.5-2 liters/day. The calcium and phosphorus homeostasis was well­maintained and she came out of dialysis after one month almost totally symptom free and with no pulmonary or lower-limb edema.

On 1 April 2006, the woman was discharged with creatinine of 330 mmol/L and phosphorus of 1.4 mmol/L to be assessed once each week. On 2 July 2006, she was re-admitted to KAUH with skin lesions that started in the gluteal re­gion. The lesions were diagnosed at the other hospital as gluteal abscess. Since the lesions were drained at the other hospital, their precise cha­racteristics were not clear.

On re-admission to our hospital, the lesions ty­pically were violaceous, painful, plaque-like, and involved the dermis and subcutaneous fat on back, buttocks, thighs, and breast [Figure 1]. Sub­sequently, the lesions progressed to ischemic/nec­rotic ulcers [Figure 2].

The patient denied a history of fever or trau­ma. On clinical examination, the following fin­dings were noted: body temperature, 37°C; blood pressure, 140/80 mmHg; jugular venous pre­ssure was not elevated and there was no lower limb edema. Laboratory tests showed creatinine of 763 mmol/L, alkaline phosphatase of 149 IU/L, calcium of 1.66 mmol/L, phosphate of 4.24 mmol/L, calcium × phosphate product of 7.0 mmol/L, parathyroid hormone of 38, ANA 1:320 g/L, dsDNA 52 iu/mL, C3 0.87 g/L, C4 0.33 g/L, CRP 130 mg/L with normal protein C, proteins, anticardiolipin and cryoglobin levels.

The patient was evaluated by dermatology and rheumatology and the lesion was biopsied. His­tology examination revealed vasculitis and pa­niculitis consistent with lupus profundus. The patient was treated with daily dialysis and oral prednisolone (0.5 mg/1 kg on alternate days) and phosphate binders. Unfortunately, the condition of the patient progressively deteriorated. The skin lesions continued to expand [Figure 3]. Conse­quently, the patient underwent surgical debride­ment of necrotic tissue of the back [Figure 4]. Staphylococcus aureus Scientific Name Search  was isolated from the skin lesions and treatment with a broad spec­trum antibiotic was started. However, the patient developed septic shock and was transferred to ICU for intubation. Based on a second skin biopsy report, calciphylaxis was diagnosed. Re­peat parathyroid hormone level after one month of this treatment was 5.3, calcium was 2.2 mmol/L, phosphate was 1.4 mmol/ L. However, despite all efforts, the patient died due to sepsis.


   Discussion Top


Calciphylaxis is a disease, which occurs as a result of deposition of calcium resulting in pain­ful skin lesions leading to advanced non-healing ulcers and skin necrosis. Calciphylaxis in he­modialysis patients was first described in 1962. It is usually seen in patients with chronic kid­ney disease with most of them being on dia­lysis. These patients usually are characterized by the presence of hypercalcemia, hyperphos­phatemia, and hyperparathyroidism. The syn­drome described above has also been reported in patients with normal kidney function as in malignancy, [2],[3] inflammatory bowel disease, [6] and primary hyperparathyroidism in association with protein C and protein S deficiency. [4],[5]

Our patient developed calciphylaxis two months after starting dialysis, with only a short duration of hyperphosphatemia, hypercalcemia, and high calcium-phosphate product. However, it is known that a long duration of hyperparathyroidism and hyperphosphatemia is crucial for the develop­ment of calciphylaxis. Mazhar et al [11] demons­trated a three-fold increase in the likelihood of developing calciphylaxis for each 1 mg/dL in­crement in serum phosphate over 12-months period prior to the diagnosis. Initially, calciphy­laxis was described in older dialysis patients although recent reports suggest that younger patients also can be at high-risk and a few pediatric cases have also been described. [12] The syndrome is common among females as in our case.

On reviewing other reports, a majority of pa­tients who developed calciphylaxis were pa­tients who had received dialysis for more than one year, and most of them were on hemo­dialysis. [13] There was an occasional report of calciphylaxis occurring in a patient on peri­toneal dialysis. We found no studies in which the incidence of calciphylaxis was compared a­mong patients who received hemodialysis or peritoneal dialysis. A recent report described two patients with SLE who developed calciphylaxis after they received peritoneal dialysis for end­stage renal disease. In one of these patients, cu­taneous lesions developed two days after ultra­violet phototherapy for pruritus. The second pa­tient was a young woman who developed cal­ciphylaxis four years after being on peritoneal dialysis. [14] In our case, calciphylaxis developed almost simultaneously with the onset of he­modialysis after only a short period of kidney failure, apparently without other causes of cal­ciphylaxis. The patient in our case was obese (BMI 37.5) and had hypoalbuminemia, both of which are strongly associated with calciphy­laxis. [15] Bleyer et al [15] reported a 17-fold increase in the risk of developing calciphylaxis with each 1 g/L reduction in serum albumin. In addition, they suggested that for an increase in BMI of 1 kg/m 2 , the odds ratio for developing calciphyla­xis was 6.29 (95% confidence interval 3.7-10.7). [15]

The mechanisms of vasculopathy in calciphy­ laxis are unclear but established risk factors in­clude disturbance of calcium-phosphate and pa­rathyroid hormone homeostasis. However, we found no reports on the underlying mechanisms for the development of calciphylaxis.

Based on this case study, it is proposed that vasculitis may be an underlying cause of calci­phylaxis. To our knowledge, this is the only case of calciphylaxis with SLE that developed at the beginning of dialysis after only a short period of impaired calcium-phosphate homeos­tasis. The patient had profound hypoalbumine­mia and she was obese which are well known risk factors for calciphylaxis. However, the po­ssibility of vasculitis as an additional risk factor should be considered.

Calciphylaxis is a lethal disease that carries a high rate of morbidity and mortality. Our pa­tient died shortly after the diagnosis of calci­phylaxis.

 
   References Top

1.Kang AS, McCarthy JT, Rownland C, Farley DR, Jon A. Is calciphylaxis best treated surgi­cally or medically? Surgery 2000;128:967-72.  Back to cited text no. 1      
2.Goyal S, Huhan KM, Provost TT. Calciphylaxis in patient without renal failure or elevated para­thyroid hormone: Possible aetiological role of chemotherapy. Br J Dermatol 2000;142:1087-90.  Back to cited text no. 2      
3.Golitz LE, Field JP. Metastatic calcification with skin necrosis. Arch Dermatol 1972;106; 398-402.  Back to cited text no. 3      
4.Bogdonoff M, Woods A, White J, Engel F. Hyper­ parathyrodism. Am J Med 1956;21:583-95.  Back to cited text no. 4      
5.Friedman M, Marshall-Jones P, Ross EJ. Cushings syndrome: Adrenocortical hyperactivity secon­dary to neoplasm arising outside the pituitary­adrenal system. QJM 1966;35:193-214.  Back to cited text no. 5  [PUBMED]    
6.Barri YM, Graves GS, Knochel JP. Calciphylaxis in patient with Crohns disease in the absence of end stage renal disease. Am J Kidney Dis 1997; 29:773-6.  Back to cited text no. 6  [PUBMED]    
7.Korkamaz C, Dundar E, Zubaroglu. Calciphyla­xis in a patient with rheumatoid arthritis without renal failure and hyperthyroidism: The possible role of long term steroid use and protein S deficiency. Clin Rheumatol 2002;21:66-9.  Back to cited text no. 7      
8.Fader DJ, Kang S. Calciphylaxis without renal failure. Arch Dermatol 1996;132:837-8.  Back to cited text no. 8  [PUBMED]    
9.Arch-Ferrer JE, Beeken SW Rue LW, Bland KI. Therapy for calciphylaxis: An outcome analysis. Surgery 2003;134:941-5.  Back to cited text no. 9      
10.Dosanjh A, Kebebew E. Calciphylaxis is usually non-ulcerating risk factors, outcome and the­rapy. J Kidney Int 2002;61:2210-7.  Back to cited text no. 10      
11.Mazhar AR, Johnson RJ, Gillen D, et al. Risk factors and mortality associated with calciphy­laxix in end-stage renal disease. Kidny Int 2001;60:324-32.  Back to cited text no. 11      
12.Zouboulis CC, Blume-Peytavi U, Lennert T, et al. Fulminant metastatic calcinosis with cutaneous necrosis in achild with end-stage renal disease and tertiary hyperparathyroidism. Br. J Dermatol 1996;135:617-22.  Back to cited text no. 12      
13.Safwan H, Sake E, Russell B, Jasin HE. Sys­temic lupus erythematosus and Calciphylaxis. J Rheumatol 2004;31:1851-3.  Back to cited text no. 13      
14.Chavel SM, Karen S, Taraska, Schaffer JV, Lazova R. Calciphylaxix associated with acute, reversible renal failure in the setting of alco­holic cirrhosis. J Am Acad Dermatol 2004;50: S125-8.  Back to cited text no. 14      
15.Bleyer AJ, Choi M, Igwemez B, de Ia, Torre E, White WL. A case control study of proximal calciphylaxis. Am J Kidney Dis 1998;32(3): 376-83.  Back to cited text no. 15      

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Correspondence Address:
Fatma I Al Beladi
Department of Internal Medicine, Nephrology Division, King Abdulaziz University Hospital, Jeddah
Saudi Arabia
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PMID: 20228522

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