Systemic Calciphylaxis Revisited

Adrogué, Horacio J.; Frazier, Mark R.; Zeluff, Barry; Suki, Wadi N.

doi:10.1159/000166536

Cited by 103 publications

(10 citation statements)

References 17 publications

(41 reference statements)

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“…Even with prompt initiation of treatment, calciphylaxis portends a dismal prognosis. The disease has a 6-month survival rate of approximately 50%, with disseminated infection continuing to account for most deaths [ 9 , 24 ]. Of note, mortality is roughly tripled among patients receiving hemodialysis, such as the patient presented here [ 4 ].…”

Section: Discussionmentioning

confidence: 99%

Calciphylaxis of the penis and distal digits: a case report

Smilnak¹,

Jiang²,

Jain³

2022

J Med Case Reports

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Background Calciphylaxis is a rare, often fatal disease resulting from calcification of dermal arterioles and capillaries. Usually diagnosed in patients with end-stage renal disease, this disorder typically presents as necrotic, nonhealing ulcers in acral or adipose areas. Here we report the case of an elderly man who was found to have calciphylaxis of the distal digits and penis, the latter of which is an uncommon site of disease that carries a particularly poor prognosis. Case presentation A 73-year-old African American man with multiple medical comorbidities including dialysis-dependent end-stage renal disease presented with worsening painful, necrotic lesions on his glans penis and several distal digits over the last 2 months. The wound on the glans was foul smelling with overlying purulence and had been unsuccessfully treated with amoxicillin–clavulanic acid. Discovery of diffuse intravascular calcification on computed tomography, in addition to a markedly elevated calcium–phosphate product immediately prior to the onset of his ulcers, led to the diagnosis of calciphylaxis. The patient was initiated on sodium thiosulfate without improvement in his lesions, and he died 3 months later after another prolonged hospitalization. Conclusions While calciphylaxis is a rare disease, involvement of the distal digits and especially the penis is even more uncommon and portends a particularly poor prognosis: 6-month mortality rates are reportedly as high as 70%. This suggests that prompt recognition and management of the disease is required; however, despite receiving standard therapy, our patient failed to experience improvement in his disease and instead developed several more fingertip ulcers at blood glucose sample points during his hospitalization. A corollary of the case presented here is the need for more effective management of calciphylaxis, especially for patients in whom uncommon sites, such as the penis, are involved.

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Section: Discussionmentioning

confidence: 99%

Calciphylaxis of the penis and distal digits: a case report

Smilnak¹,

Jiang²,

Jain³

2022

J Med Case Reports

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“…Utilizing these two steps he induced metastatic systemic calcification; however, vascular calcification was not observed [11]. In the late 1960s, peripheral ischemic tissue necrosis, vascular calcification, and cutaneous ulcerations were reported in patients on dialysis or after renal transplantation mainly in cases of severely advanced renal HPT [1, 2]. The syndrome resembled that seen in Selye’s model, and was termed uremic calciphylaxis .…”

Section: Discussionmentioning

confidence: 99%

“…It was suggested that the skin lesion induced by systemic vasculitis or hypercoagulability without vascular calcification should be excluded from the uremic calciphylaxis syndrome [6]. It is not yet clear whether the clinical manifestations induced by vascular calcification (i.e., ischemic heart disease, ischemic colitis, lung calcification) without a skin necrotic lesion are actually involved in the uremic calciphylaxis syndrome, which occurs in patients on or before HD and after successful kidney transplantation [1, 12]. Calciphylaxis progresses to the internal organs, involving the lung, myocardium, and intestine, and is frequently complicated with ischemic heart disease and/or valvular disease [14].…”

Section: Discussionmentioning

confidence: 99%

“…The lesions are divided into two types, proximal and distal. In the former, the lesions involve predominantly skin of the upper thigh, chest, breast, abdominal wall, buttock, penis, and scrotum; in the latter, the lesions occur below the knees, and on the fingers [1, 2]. The histopathological characteristics change, and in the skin biopsies calcium deposits are seen within arteriole‐sized and small vessel walls, with endovascular fibrosis associated with fat necrosis and calcification, as well as infiltration of neutrophils and macrophages.…”

mentioning

confidence: 99%

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Calciphylaxis: A Rare Complication of Patients Who Required Parathyroidectomy for Advanced Renal Hyperparathyroidism

et al. 2005

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Calciphylaxis is a relatively rare but life-threatening complication in uremic patients. Clinical findings and prognosis were evaluated in six patients who developed calciphylaxis from a group of 1499 patients who underwent parathyroidectomy (PTx) for advanced renal hyperparathyroidism (HPT) in our department from July 1972 to July 2003. The frequency of calciphylaxis was 0.40% (6/1499). Two patients were women and four were men. The mean age was 50.5 years, and the mean duration of hemodialysis (HD) treatment was 14.0 years. In five of six patients, calciphylaxis was classified as distal type; in one case, as proximal type. In three patients, calciphylaxis was diagnosed at the time for PTx. In two patients, calciphylaxis was identified after PTx, although the serum parathyroid hormone (PTH) level was within the appropriate range for dialysis patients. In two patients, calciphylaxis improved after PTx, but two patients required leg and toe amputations after PTx. In one patient with the proximal type of calciphylaxis, the condition occurred when a high PTH level recurred after the initial PTx. The patient died as a result of a serious infection due to uncontrollable skin ulcers. Calciphylaxis is a rare complication in patients who require PTx for renal HPT. Especially the proximal type has a poor prognosis. High levels of the Ca x P product and/or PTH are risk factors. Therefore, this syndrome should be kept in mind and attention should be paid to reduce risk factors. It is important that PTx being performed at the right time in patients with advanced renal HPT refractory to medical treatment.

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“…Calciphylaxis is an uncommon disease, occurring in < 5% of dialysis patients[4] but carries a dismal prognosis, with the estimated 1-year survival rate below 50%,[5] with death occurring most frequently from infection. [6] In a retrospective series,[7] the most commonly affected site was the legs, affecting 60% of patients, followed by the abdomen and buttocks. Females are more commonly affected.…”

Section: Case Reportmentioning

confidence: 99%

The role of bone scintigraphy with single-photon emission computed tomography-computed tomography in the diagnosis and evaluation of calciphylaxis

2017

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Calciphylaxis, also known as calcific uremic arteriolopathy, is an uncommon disease, typically found in patients with the end-stage renal disease. Pathophysiological features include small vessel vasculopathy with mural calcification, fibrosis, and thrombosis. The clinical presentation varies but often consists of the necrosis of skin and subcutaneous tissues, with or without visceral organ involvement due to small vessel vasculopathy. This condition has a significant morbidity and mortality, making accurate diagnosis imperative. We present a case of calciphylaxis investigated using planar bone scintigraphy and single-photon emission computed tomography-computed tomography (SPECT-CT). This case stresses the value of SPECT-CT to accurately localize the abnormal uptake in subcutaneous soft tissue microcalcifications and confirms the exact location and extent of pathology.

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Systemic Calciphylaxis Revisited

Cited by 103 publications

References 17 publications

Calciphylaxis of the penis and distal digits: a case report

Calciphylaxis of the penis and distal digits: a case report

Calciphylaxis: A Rare Complication of Patients Who Required Parathyroidectomy for Advanced Renal Hyperparathyroidism

The role of bone scintigraphy with single-photon emission computed tomography-computed tomography in the diagnosis and evaluation of calciphylaxis

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