<p>Calciphylaxis: An Analysis of Concomitant Factors, Treatment Effectiveness and Prognosis in 30 Patients</p>

Abstract

Background: Calciphylaxis is a rare but severe complication mostly affecting patients with end-stage renal disease (ESRD) and is associated with high morbidity and mortality. The natural history, concomitant factors, pathogenesis, and treatment for calciphylaxis remain equivocal. Methods: We conducted a retrospective study on patients diagnosed with calciphylaxis in a tertiary care center between January 1, 2012, and December 31, 2017. We describe demographics, co-morbidities, laboratory parameters, effectiveness of sodium thiosulfate treatment and outcomes. Results: Of the 30 patients (age 65.6 ± 12.79 years, male:female = 8:22), 23 (76.67%) had ESRD and were either on hemodialysis (15 [65.22%], median duration 22.5 months [range 0.2-96 months]) or peritoneal dialysis (8 [34.78%], duration 29±10 months). Predisposing home medications: 8 (28%) had calcium supplements, 10 (36%) had warfarin, 16 (57%) had vitamin D and 5 (18%) had iron supplements. The median parathyroid hormone (PTH) level was 239.8 pg/mL (range 4.7-2922). Calciphylaxis was found on extremities in 21 (70%) and on torso in 6 (20%) patients. Sodium thiosulfate (STS) was given for treatment in 20 (67%) patients and 3 were cured in <2.25 months. One-year survival for all patients with calciphylaxis was 26% (29% for STS group and 20% for those that did not receive STS) and following any surgical treatment regardless of STS use was 14%. Limitations: Retrospective design, absence of a control group and low power. Conclusion: Calciphylaxis was more common among females with a predilection for extremities over the torso. Elevations in PTH and inflammatory markers were common. Treatment with STS did not show a statistically significant improvement in survival. Those who were cured, were treated with STS up to three months.

Therapeutic effect of intravenous sodium thiosulfate for uremic pruritus in hemodialysis patients

“…Skin disorders associated with HD can markedly affect patients’ quality of life and can negatively impact their mental and physical health [ 21 ]. CUA is triggered by an imbalance in the promoters and inhibitors of vascular calcification caused by the inflammatory changes that occur in uremia [ 22 ]. STS has been shown to improve skin lesions caused by calciphylaxis [ 23 , 24 ].…”

The Role of Iron in Calciphylaxis—A Current Review

“…It is a very serious and frequently fatal condition of cutaneous microvascular calcification and thought to be an independent risk factor linked to all-cause mortality [ 2 , 3 ]. Although rare, it is most commonly documented in patients with chronic kidney disease (CKD), in particular those with end-stage renal disease (ESRD), affecting up to 1–4% of ESRD patients, associated with high morbidity and mortality [ 4 , 5 , 6 ] Once diagnosed, prognosis is often poor, even with treatment, with an associated mortality of around 45–80% at one year [ 2 , 7 ]. CKD patients are at increased risk for developing vascular calcification due to their associated uraemia which predisposes to inflammation and endothelial dysfunction, although it has rarely been reported in patients without evidence of renal dysfunction, termed non-uraemic CUA [ 2 , 8 , 9 ].…”

“…Although the exact pathogenesis of CUA is undetermined, it is thought to result from extensive microvascular calcification and thrombosis within the media of arterioles, occlusion of which can result in necrosis of the overlying skin and soft tissues [ 5 , 6 , 8 ]. The precise pathophysiology of microvascular calcification is unclear; it is suggested that there is likely an underlying imbalance between promoters and inhibitors of vascular calcification in those who develop this condition [ 8 ].…”

“…The precise pathophysiology of microvascular calcification is unclear; it is suggested that there is likely an underlying imbalance between promoters and inhibitors of vascular calcification in those who develop this condition [ 8 ]. Numerous risk factors are suggested in its development; namely ESRD, female gender, Caucasian ethnicity, diabetes mellitus, obesity or significant rapid weight loss, local trauma, abnormalities in coagulation such as protein C or S deficiency, autoimmune disorders, recurrent hypotension, hyperparathyroidism, hypercalcaemia, hyperphosphataemia, elevated alkaline phosphatase, hypoalbuminaemia and exposure to ultraviolet light [ 2 , 5 , 7 , 8 , 9 , 12 , 13 ]. Additionally, there are several medications which have been associated with increased risk, such as, vitamin K antagonists, calcium, calcium-containing phosphate binders, phosphate, erythropoiesis-stimulating agents (ESAs), iron, vitamin D supplements and immunosuppressants, such as methotrexate and corticosteroids [ 5 , 6 , 7 , 12 ].…”

“…Numerous risk factors are suggested in its development; namely ESRD, female gender, Caucasian ethnicity, diabetes mellitus, obesity or significant rapid weight loss, local trauma, abnormalities in coagulation such as protein C or S deficiency, autoimmune disorders, recurrent hypotension, hyperparathyroidism, hypercalcaemia, hyperphosphataemia, elevated alkaline phosphatase, hypoalbuminaemia and exposure to ultraviolet light [ 2 , 5 , 7 , 8 , 9 , 12 , 13 ]. Additionally, there are several medications which have been associated with increased risk, such as, vitamin K antagonists, calcium, calcium-containing phosphate binders, phosphate, erythropoiesis-stimulating agents (ESAs), iron, vitamin D supplements and immunosuppressants, such as methotrexate and corticosteroids [ 5 , 6 , 7 , 12 ]. In a previous study it was found that vitamin K antagonist use, high C-reactive protein, low albumin and low haemoglobin in association with CUA were markers of poor prognosis similar to observations from a French cohort [ 3 , 14 ].…”