Calciphylaxis: Beyond CKD-MBD. / Calcifilaxis: más allá de CKD-MBD.

INTRODUCTION: Calcific uraemic arteriolopathy (CUA), also called calciphylaxis, is a rare but potentially fatal vascular disorder that almost exclusively affects patients with chronic renal failure. The objective of this study was to analyse various risk factors for developing CUA and its subsequent clinical course according to the treatment received. MATERIALS AND METHODS: A retrospective study that included patients diagnosed with CUA from December 1999 to December 2015. Various risk factors, clinical course and treatment options were analysed. RESULTS: A total of 28 patients (53.6% females) with a mean age of 67.2±11.8 (38-88) years were included. At the time of diagnosis, 53.6% were on haemodialysis, 25% were kidney transplant patients and 21.4% had normal renal function. The use of steroids (100%, P=.001) was the main risk factor in renal transplant patients. Skin lesions resolved in 60.7% (especially in those receiving multitargeted therapy). Patient survival at 12 months was 29% in transplant patients, 57% in haemodialysis patients and 100% in normal renal function patients (log-rank 6.88, P=.032). Chronic renal failure (P=.03) and hypoalbuminaemia (P=.02) were the main risk factor for CUA mortality. CONCLUSIONS: Although the incidence of CUA remains low, CUA mortality is very high, Special attention to its occurrence in kidney transplant patients and «non-renal¼ CUA forms is required. Oral anticoagulants and steroids appear to be the main risk factors, CUA is a challenge; a registry of patients and determining standard therapy are required.

Survival, Risk Factors, and Effect of Treatment in 101 Patients With Calciphylaxis.

OBJECTIVE: To report on the survival and the associations of treatments upon survival of patients with calciphylaxis seen at a single center. PATIENTS AND METHODS: Using the International Classification of Diseases, Ninth Revision diagnosis code of 275.49 and the keyword "calciphylaxis" in the dismissal narrative, we retrospectively identified 101 patients with calciphylaxis seen at our institution between January 1, 1999, through September 20, 2014, using a predefined, consensus-developed classification scheme. RESULTS: The average age of patients was 60 years: 81 (80.2%) were women; 68 (68.0%) were obese; 19 (18.8%) had stage 0 to 2 chronic kidney disease (CKD), 19 (18.9%) had stage 3 or 4 CKD; 63 (62.4%) had stage 5 or 5D (dialysis) CKD. Seventy-five patients died during follow-up. Six-month survival was 57%. Lack of surgical debridement was associated with insignificantly lower 6-month survival (hazard ratio [HR]=1.99; 95% CI, 0.96-4.15; P=.07) and significantly poorer survival for the entire duration of follow-up (HR=1.98; 95% CI, 1.15-3.41; P=.01), which was most pronounced in stage 5 or 5D CKD (HR=1.91; 95% CI, 1.03-3.56; P=.04). Among patients with stage 5/5D CKD, subtotal parathyroidectomy (performed only in patients with hyperparathyroidism) was associated with better 6-month (HR=0.12; 95% CI, 0.02-0.90; P=.04) and overall survival (HR= 0.37; 95% CI, 0.15-0.87; P=.02). CONCLUSION: Calciphylaxis is associated with a high mortality rate. Significantly effective treatments included surgical debridement and subtotal parathyroidectomy in patients with stage 5/5D CKD with hyperparathyroidism. Treatments with tissue-plasminogen activator, sodium thiosulfate, and hyperbaric oxygen therapy were not associated with higher mortality.

Hyperbaric oxygen in the treatment of calciphylaxis: A case series and literature review.

AIMS: Calcific uraemic arteriolopathy (CUA) or calciphylaxis is most commonly seen in end-stage renal disease and is associated with significant morbidity and mortality. The aim of this study was to determine whether hyperbaric oxygen therapy (HBOT) is effective in healing calciphylaxis lesions and to determine if there are any patient factors that can predict wound healing and patient survival. METHODS: We identified by retrospective review all cases of CUA referred to our institution for treatment with HBOT. We documented the clinical and biochemical parameters of this patient population, the size and distribution of the lesions as well as wound outcomes and patient survival following treatment. RESULTS: A total 46 patients were identified with CUA associated with renal failure. Of the 46 patients, only 34 received a full course of HBOT. The balance was deemed unsuitable for treatment or was unable to tolerate treatment and was palliated. Of the 34 patients that received a full course of HBOT, 58% showed improvement in their wound scores, with more than half of these patients having complete healing of their wounds. The balance did not benefit from the therapy and had a very poor prognosis. Those that benefited from HBOT survived on average for more than 3 years. The only factor significantly associated with improved wound healing and survival was diabetes. CONCLUSION: This retrospective analysis suggests a role for HBOT in the treatment of CUA with more than half of the treated patients benefiting and surviving for an average of more than 3 years.

[Calciphylaxis: an enigma to the nephrologist]. / Calcifilassi: un enigma per il nefrologo.

Calcific uremic arteriopathy (CUA), also known as calciphylaxis, is a rare condition occurring in patients with moderate to severe chronic kidney disease. It is a serious, debilitating and potentially fatal clinical disorder affecting 1-4% of the dialysis population and is associated with a high mortality rate (60-80%). The clinical picture is characterized by painful skin lesions tending to necrotic or gangrenous ulceration ultimately necessitating amputation. Severe infectious complications leading to sepsis and death are frequent. The pathogenesis of CUA is still unknown and several pathogenetic hypotheses have been put forward; this makes its treatment difficult and often empirical. The current paper presents a systematic review of recent findings on the pathogenesis, diagnosis and treatment of CUA.

Calciphylaxis: no therapeutic concepts for a poorly understood syndrome?

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.

Calciphylaxis in chronic renal failure.

Calciphylaxis is a rare and life-threatening complication that is estimated to occur in 1% of patients with ESRD each year. Typically, extensive microvascular calcification and occlusion/thrombosis leads to violaceous skin lesions, which progress to nonhealing ulcers and sepsis. Secondary infection of skin lesions is common, often leading to sepsis and death. The lower extremities are predominantly involved (roughly 90% of patients). Patients with skin involvement over the trunk or proximal extremities have a poorer prognosis. Although most calciphylaxis patients have abnormalities of the calcium:phosphate axis or elevated levels of parathyroid hormone, these abnormalities do not appear to be fundamental to the pathophysiology of the disorder, and the etiology of calciphylaxis remains unclear. Recently, functional protein C deficiency has been hypothesized to cause a hypercoagulable state that could induce thrombosis in small vessels, with resulting skin ischemia, necrosis, and gangrene. The lack of understanding of the pathophysiology of the disease results in treatments that are equally unsatisfactory. Patients who undergo parathyroidectomy have a tendency to improve, but the prognosis for the disease is poor and mortality remains high.