Year : 1994 | Volume
: 5 | Issue : 3 | Page : 374--378
Natural progression of calciphylaxis in a patient on haemodialysis
Abraham George1, T Timothy Paul2, Chandrahasan Kedharnath1, Magdy Diwan1, Mohammad Ashraf Want1, Hla Mon1,
1 Department of Nephrology, King Fahd Central Hospital, Gizan, Saudi Arabia
2 Saudi Center for Organ Transplantation, Riyadh, Saudi Arabia
King Fahad Central Hospital, P.O. Box 204, Gizan
A 40 year old patient with end-stage renal disease developed features of calciphylaxis and tumoral calcification after four and half years on regular haemodialysis. The patient had hyperphosphatemia, with high calcium-phosphate index and radiological evidence of hyperparathyroidism before the onset of symptoms. Conservative management was without success and the patient developed florid symptoms. Parathyroidectomy was delayed due to unavoidable circumstances. When it was eventually performed there was a prompt regression of the calcific masses, but not of the vascular calcifications. The natural progression of the disease and its regression by parathyroidectomy provides an interesting insight into this phenomenon, the full blown picture of which is rarely seen these days.
|How to cite this article:|
George A, Paul T T, Kedharnath C, Diwan M, Want MA, Mon H. Natural progression of calciphylaxis in a patient on haemodialysis.Saudi J Kidney Dis Transpl 1994;5:374-378
|How to cite this URL:|
George A, Paul T T, Kedharnath C, Diwan M, Want MA, Mon H. Natural progression of calciphylaxis in a patient on haemodialysis. Saudi J Kidney Dis Transpl [serial online] 1994 [cited 2020 Apr 2 ];5:374-378
Available from: http://www.sjkdt.org/text.asp?1994/5/3/374/41162
Extra-skeletal calcifications including calcification of arteries, periarticular tumoral calcification and visceral calcifications are well known in end-stage renal disease (ESRD) patients ,,, . Calciphylaxis is characterized by peripheral ischemic necrosis and may involve tissues like tips of fingers, toes, ankles, thighs, buttocks etc. , . The exact pathogenesis of calciphylaxis is not known, but vasospasm related to high serum levels of calcium or parathyroid hormone have been implicated , . Elevated calcium-phosphate product is believed to be one of the major factors responsible for tumoral calcification and could be reversed by decreasing plasma phosphate level by drug therapy as well as dietary restriction of phosphate , . The occurrence of calciphylaxis invariably necessitates parathyroidectomy ,, . Parathyroidectomy yields prompt results in such cases non-responsive to conservative measures ,, .
A 40 year old male patient with ESRD developed polyarticular symptoms nearly four and half years after being on regular maintenance haemodialysis. Symptoms started as migrating polyarthralgia with minimal swelling relieved by non-steroidal anti-inflammatory drugs. Six months later he developed swelling over the mid-tarsal region of both feet and dorsum of the left second metacarpal bone. Later, he developed swelling of right fifth metatarsal bone followed by cystic swelling of volar and radial aspects of the left wrist, dorsum of the right wrist, ulnar side and back of the left elbow, lateral aspect of the left knee, over the mid-dorsal spine and right sterno-clavicular joint. Associated with the above symptoms, he developed evidence of gangrene involving the tips of fingers and toes and ulceration of the tumorous calcification over the volar aspect of the left wrist. Skeletal survey showed extensive calcification involving the above mentioned sites associated with arterial calcification. [Figure 1],[Figure 2],[Figure 3],[Figure 4] show photographs of the calcific masses and [Figure 5] shows disappearance of the lesion in one site following parathyroidectomy. [Figure 6],[Figure 7],[Figure 8],[Figure 9],[Figure 10] show radiological evidence of peri-articular calcifications and [Figure 11],[Figure 12] show disappearance of the lesion following parathyroidectomy.
Biopsy of one of the cystic lesions from the wrist showed on gross examination, an yellowish brown mass of 25 x 20 x 8 mm whose external surface was granular. Cut surface showed a cavity with smooth yellowish lining with a wall thickness of 2 mm. Microscopy showed large areas of calcium deposition in the form of spherules, flakes or granules surrounded by intense foreign body cell reaction and fibrosis. The blood vessels showed thrombosis and occlusion of the luminae.
The patient was on calcium carbonate tablets 600 mg thrice daily, aluminum hydroxide capsules 400 mg thrice daily and 1-a hydroxyl vitamin D3 0.25 μgm once daily, before the onset of illness. The dose of aluminum hydroxide was stepped up to 800 mg thrice daily after the onset of tumorous calcification but this measure did not influence the course of his illness. The monthly serum calcium levels till the evolution of tumoral calcification averaged 2.46 mmol/1 with a range of 2.28 to 2.70 mmol/1 (normal 2.25 to 2.75 mmol/1). The serum phosphorous averaged 2.24 mmol/1 with a range of 2.22 to 4.08 mmol/1 (normal 0.8 to 1.5 mmol/1) and serum alkaline phosphatase showed a mean of 298.12 IU with a range of 236 to 247 IU (normal 25 to 97 IU). Parathyroid hormone assay could not be done due to lack of facilities. CT scan and ultrasound of the parathyroid gland did not reveal any adenoma. Patient underwent elective parathyroidectomy nearly 10 months after the onset of tumorous calcification. His calcium and phosphorous levels immediately prior to parathyroidectomy were 2.58 mmol/1 and 2.7 mmol/1 respectively. Subtotal parathyroidectomy with preservation of part of a single parathyroid gland was performed. Post-operatively his serum calcium level came down to 1.2 mmol/1 and was managed by administration of intravenous calcium. During the subsequent course, his symptoms steadily improved with complete resolution of tumorous calcification and ischemic gangrene. However, full blown gangrene of his right second toe necessitated an amputation. At the time of reporting the patient continues to be on regular haemodialysis at our center and since the low serum calcium levels have persisted, he is on treatment with high dose 1-a hydroxy vitamin D3 along with calcium carbonate supplementation.
Calciphylaxis and tumoral calcification are well known complications of ESRD ,,, . Parathyroidectomy is a well established form of treatment for patients with such complications non-responsive to conventional treatment , . Hyperphosphatemia was difficult to control in this case possibly due to non-compliance to phosphate binders and failure to restrict dietary phosphate. Without decreasing the serum phosphorous levels, increasing the dose of vitamin D derivatives or calcium carbonate may further adversely affect the calcium-phosphate index  . Hence the choice was mainly limited to surgery in this case, especially in view of calciphylaxis. Parathyroidectomy was delayed for 10 months due to the lack of facilities for this surgery at the treating center as well as due to some unavoidable circumstances. Nonetheless, the situation provided an opportunity to study the natural progression of metastatic calcification and calciphylaxis unresponsive to conservative management. Parathyroidectomy promptly corrected the tumoral calcification and calciphylaxis. Also, there was evidence of bone remodeling and healing. However, vascular calcifications were found to persist in this case despite parathyroidectomy and such lesions are known to be resistant to parathyroidectomy  .
We are grateful to Regina Stephen for the excellent secretarial help and Mohd Farooque Khan for the able technical assistance rendered.
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