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Calciphylaxis is a rare, potentially life-threatening syndrome characterized by progressive microvascular and superficial soft tissue calcification, usually seen in patients with chronic renal failure. We describe an unusual case of calciphylaxis in a patient with alcoholic liver disease and normal renal function who responded well to conservative wound care.
Calciphylaxis is a dreaded complication of renal failure characterized by nodular subcutaneous calcification and painful tissue necrosis often leading to ulceration, secondary infection, and high mortality rates. The case of a woman receiving continuous ambulatory peritoneal dialysis who had a typical clinical presentation of calciphylaxis confirmed by x-ray and technetium scan findings is described. After nonresponse to conventional therapy, treatment with intravenous sodium thiosulfate 3 times weekly was begun, and she had rapid and dramatic relief of signs and symptoms and improvement of technetium scans. Prolonged treatment was well tolerated without serious side effects and accompanied by continued clinical improvement.
INTRODUCTION: Calciphylaxis (calcific uremic arteriolopathy) is a rare complication of end-stage renal disease in patients with secondary hyperparathyroidism. Subcutaneous skin nodules and extensive tissue necrosis with vascular calcification characterize this process. DESIGN AND SETTING: The design of the study included case series and literature review. The study was set in a tertiary care hospital. PATIENTS: Four patients presented over an 8-month period with extensive tissue loss and the subsequent diagnosis of calciphylaxis. Two of these patients were revascularized. One underwent primary amputation, and the final patient died before revascularization. The mortality rate for this group was 75%. All patients had significant vascular complications outside the area of initial presentation. CONCLUSIONS: These individuals represent a subset of patients who may not benefit from revascularization or may require extensive regulation of divalent metabolism before consideration for revascularization.
Calcific uremic arteriolopathy (or calciphylaxis) is a severe complication of renal failure characterized by subcutaneous calcification of the small arteries and tissue necrosis. We describe the case of a woman receiving continuous cycling peritoneal dialysis with calciphylaxis involving upper and lower extremities. After intolerance of intravenous sodium thiosulfate and limited intravenous access options, we administered sodium thiosulfate intraperitoneally and quantitated the amount of extra calcium removed. Intraperitoneal administration of sodium thiosulfate was well tolerated and led to removal of extra calcium with peritoneal dialysis.
A 42-year-old woman underwent hemodialysis secondary to diabetic nephropathy. Total parathyroidectomy with forearm autograft was performed due to secondary hyperparathyroidism (HPT) complicated with calciphylaxis. Rapidly progressive enlargement of autograft with unusual "gourd-shape" developed, and then it was removed. Pathologic examination of the autograft disclosed multinodular hyperplasia. Residual parathyroid gland in the retrothyroid region was found later. Rapidly recurrent HPT originating from both the residual parathyroid tissues and the enlarged autograft within such short time after parathyroidectomy is rare in the literature. The multinodular hyperplasia pattern of the parathyroid gland may be a major factor for such rapid recurrence. In addition to good control of calcium and phosphate, regular follow-up of parathyroid hormone level and imaging studies of not only autografted gland at the forearm but also possibly residual parathyroid tissues at the neck are important for monitoring recurrence in maintenance hemodialysis patients after parathyroidectomy with forearm autograft, especially in those with pathologic type of nodular hyperplasia and calciphylaxis.
This is the first report on tandem cryofiltration apheresis (CFA) and hemodialysis (HD). A 44 year old white man with Type II mixed cryoglobulinemia, hepatitis C virus (HCV), severe skin lesions, and end-stage renal disease (ESRD) on maintenance hemodialysis was air-transferred for CFA, which is only available at our medical center. The patient failed to respond to high dose steroids, immunosuppression, intravenous immunoglobulin (IVIG), and plasma exchange for the treatment of his cryoglobulinemia, and he failed alpha-interferon therapy for his HCV. On arrival, he was also found to have severe calciphylaxis secondary to ESRD with generalized, painful skin ulceration, necrosis, and penile gangrene. To treat both conditions, intensive, tandem CFA/HD was initiated. He received extensive wound care and surgical debridement. To prevent pressure ulcers and worsening of skin lesions, he was placed on the FluidAir (Kinetic Concepts Inc., San Antonio, TX) controlled air bed. The patient received 18 tandem CFA/HD treatments, and four extra HD treatments in one month. Sodium citrate was used as an anticoagulant for the CFA procedure. His plasma cryoglobulin (CG) level dropped from 6,157 to 420 microg/ml, and his calciphylaxis also improved. The CFA effectively removed 93% of CG, without significant removal of IgG, IgM, IgA, albumin, and fibrinogen. No albumin or fresh frozen plasma (FFP) was required as replacement fluid for CFA. No citrate toxicity or evidence of complement activation with the cryofilter was observed. The entire CFA procedure time (3(1/2) hours) was considered. Intensive, tandem CFA/HD was performed in a critically ill patient with no apparent adverse consequences.
There is mounting evidence that elevated serum phosphorus is an important cardiovascular risk factor in patients with end stage renal disease. Recent work has shown that vascular smooth muscle cells have the ability to undergo osteoblastic differentiation and produce an environment conducive to mineralization. Serum phosphorus is an important stimulator of this process and the adverse cardiovascular effects of hyperphosphatemia are most likely mediated via its ability to enhance the development of vascular calcification. Arterial calcification, whether it is intimal or medial in location, is a strong independent risk factor for cardiovascular morbidity and mortality. Both coronary artery calcification and calciphylaxis are prototypical examples of arterial calcification that have been associated with poor phosphate control. Furthermore, several investigators have recently suggested that the prescription of large doses of calcium to achieve phosphate control may augment, rather than diminish, the risk of vascular calcification. This is more likely to be true in the presence of low turnover bone disease, a diagnosis difficult to make with routine laboratory testing. A brief review of the molecular biology of vascular calcification supports the concept that warfarin administration may exacerbate the calcific process, particularly in the setting of hyperphosphatemia, as has been reported in patients with calciphylaxis. Recognizing the consequences of poor phosphate control, it is time to adopt strict target levels that aim to normalize serum phosphorus levels. The available evidence supports that this control should not be achieved through the use of supraphysiologic doses of supplemental calcium.
Calciphylaxis is a highly morbid syndrome of vascular calcification and skin necrosis, the pathophysiology of which remains largely elusive. We report a patient with end-stage renal disease and multiple painful skin lesions who underwent a bone scan for extremity pain. Increased tracer accumulation was seen in the subcutaneous tissues of the trunk and lower extremities. In this case, the bone scan aided in the diagnosis and treatment of calciphylaxis for a patient who experienced a relatively short hospital stay.
Calciphylaxis is a condition with a high mortality rate that is often found in patients with renal failure. It is characterized by soft tissue calcification and painful skin ulceration. A serum calcium-phosphorus product of more than 60 mg2/dl2 indicates great risk for calciphylaxis. The diagnosis is made on the basis of an incisional biopsy showing calcification of the small, subcutaneous arteries.
A 50-year-old Japanese female with chronic renal failure who had been on continuous ambulatory peritoneal dialysis developed fulminant systemic cutaneous necrosis that began as painful livedo reticularis-like skin lesions on her thighs. Because of disseminated vascular calcification within the muscular layer of her lower limbs, we eventually diagnosed her with calciphylaxis. The skin necrosis progressed rapidly, and she died of sepsis and pneumonia on the 53rd hospital day. In addition to her long-lasting severe hyperparathyroidism and extremely elevated serum phosphorus and calcium levels, mechanical, frictional stimulation inflicted on the local skin and administration of corticosteroids were suspected to have precipitated the calciphylaxis. Our lack of awareness of this disease in its early stages resulted in our missing the chance to do a parathyroidectomy that might have changed the course. It is important to know the clinical features of this rare disease in order to make a diagnosis as early as possible.
Calciphylaxis is a rare and life-threatening condition of progressive cutaneous necrosis secondary to small and medium-sized vessel calcification. It is seen almost exclusively in patients with end-stage renal disease and secondary hyperparathyroidism. We experienced a case of 67-year-old man with calciphylaxis that manifested with characteristic skin lesions, pathologic findings, and laboratory changes. His skin lesions began as painful erythematous patches and subsequently progressed to necrotic ulcers with eschars on the distal aspect of the extremities. Pathologically, calcification was found in small and medium-sized blood vessels in the deep dermis and subcutaneous tissue. His serum calcium was 9.5 mg/dL, phosphorus was 7.8 mg/dL, and nPTH was 99.9 pg/mL. The patient had been treated with surgical debridement and other supportive treatment. However, he eventually underwent an amputation below the right knee and died from sepsis.
Calciphylaxis is a rare but significant condition. It is associated with a high degree of morbidity and is fatal in between 60% and 80% of patients. It occurs most commonly in patients with endstage renal failure and is associated with hypercalcaemia or hyperphosphataemia or both (elevated calcium-phosphate product). Secondary hyperparathyroidism is also common. Clinically, patients develop rapidly progressive, necrotic skin ulcers that are extremely painful. They, universally, respond poorly to usual ulcer therapies. Some surgeons advocate parathyroidectomy for patients with calciphylaxis. Evidence is inconclusive regarding this treatment; however, some trials have shown improved rates of ulcer healing and overall survival in patients treated with parathyroidectomy.
'Calciphylaxis', a calcification syndrome associated with ischaemic cutaneous necrosis, is acquired naturally in humans in disease states. It is a life and limb-threatening complication, usually observed in patients with renal disease and secondary hyperparathyroidism, but known to occur in the absence of renal or parathyroid disease. The reported mortality rate, which ranges from 60-80%, relates to wound infection, sepsis and organ failure. It is a small-vessel vasculopathy, which is estimated to occur in about 4% of haemodialysis patients. Clinically, violaceous, reticulate areas of cutaneous necrosis and eschar may be evident, particularly in the extremities. In addition to the clinical picture, a raised calcium phosphorous product, an elevated parathyroid hormone level, radiographic evidence of vessel and soft-tissue calcification and the finding of mural calcification affecting small arteries and arterioles on histopathology help to confirm the diagnosis of this entity which generally has a poor prognosis. A high index of suspicion and an active multidisciplinary management approach, with rigorous attention to wound care and prevention of sepsis, are vital in the management of these patients. In this overview, we discuss the pathophysiology, clinical features and associations, risk factors, diagnosis and management issues relating to calciphylaxis.
Calciphylaxis is a very uncommon and severe disease which mainly appears in patients with chronic renal insufficiency. It presents with ischemia and necrosis of the skin, subcutaneous adipose tissue, muscles and rarely viscera. The pathogenetic mechanisms inducing calciphylaxis are for the most part unknown. The mortality rate of 80% in the first year is very high. Patients experience marked pain, recurrent infections and the constant risk of secondary sepsis. Even multidisciplinary therapeutic strategies are limited, although there are recent case reports providing promising new therapeutic options including sodium thiosulfate and cinacalcet. This review summarizes the important aspects of diagnosis, pathogenesis, prevention and the possible therapeutic strategies of this intriguing, rare and often fatal disease.
A renal transplant patient who presented with a painful, proximal myopathy due to systemic calciphylaxis is described. The myopathy preceded the characteristic skin and soft tissue necrosis. Systemic calciphylaxis should be considered in a dialysis or a renal transplant patient presenting with a painful proximal myopathy even in the absence of necrotic skin lesions.
Calciphylaxis is a rare but frequently fatal complication in patients with end-stage renal disease. Original concepts regarding groups at risk for the disease, predisposing factors, and associated morbidity have changed significantly in the past few years as more cases are reported. We present a patient who developed fatal calciphylaxis in the setting of moderate renal insufficiency to illustrate some of the evolving concepts in this disease process.
Calciphylaxis is a rapidly developing, fatal process of vascular calcium deposition with prominent cutaneous manifestation. We treated a long-term haemodialysis patient who developed an analogous disorder limited to the lungs. A 57-year-old man was admitted for initiation of peritoneal dialysis because limited cardiac reserve precluded further haemodialysis. He was treated successfully for pneumonia until hypoxia and progressive hypercalcaemia developed. (99m)Tc-methylene disphosphonate scintigraphy showed diffusely increased pulmonary uptake. Death supervened despite aggressive and successful treatment of hypercalcaemia. Autopsy studies included immunohistochemistry and morphometric studies of bone. Alveolar capillary walls showed diffuse calcium deposition. Both gross and microscopical findings differed from those of typical metastatic calcification in dialysis patients. Immunoreactivity for parathyroid hormone-related protein was present in the lesions. Bone histomorphometry indicated mild osteitis fibrosa. Pneumonia is believed to have caused local synthesis of parathyroid hormone-related protein that, along with high calcium x phosphorus product, contributed to calcium deposition. By analogy with the cutaneous process we termed the deposition "pulmonary calciphylaxis".
A 35-year-old haemodialysis-dependent woman with chronic renal failure developed large, very painful necrotic ulcers and necrosis on the thighs, buttocks and the abdomen with signs of fast progression. The skin biopsy specimens showed a broad necrosis of the epidermis and thrombosed dermal vessels with focal calcium deposits within the wall. In addition, laboratory findings presented an increased product of serum calcium and phosphate concentrations. Thus, we diagnosed calciphylaxis on the basis of clinical, biochemical and histopathological criteria. We initiated a therapy in which our patient was treated with intravenous sodium thiosulphate 3 times weekly. Already after 2 weeks of treatment, no new lesions were detectable and there was a dramatic pain relief. In the following 4 weeks, a successive decline of the ulcers and the healing of individual tissue defects could be seen. Four months after the start of the therapy, the patient underwent successful renal transplantation. Thus, the intravenous therapy of calciphylaxis with sodium thiosulphate might be a new effective alternative in the treatment of this life-threatening disease.