[Calciphylaxis, risk factors, treatment and outcomes. Retrospective study of 39 patients]. / Calcifilaxis, factores de riesgo, tratamiento y evolución. Estudio retrospectivo de 39 pacientes.

INTRODUCTION: Calciphylaxis is a serious vascular disorder characterized by calcification of tunica media, intimal hyperplasia, thrombosis, and skin necrosis. It was described in patients with renal failure (UC), although it can occur in its absence (NUC). Its risk factors are under study and its diagnosis can be complex. Over a decade ago, its mortality was estimated at 60-80%. Recent studies indicate that it has decreased (40%). METHODS: A retrospective study was carried out in the period between January 1, 2011 and December 31, 2019. The past medical record, clinical characteristics, laboratory and histopathological findings, and evolution of all patients with calciphylaxis evaluated at the Hospital Italiano de Buenos Aires were reviewed. RESULTS: Thirty-nine patients were included. Sixtyone percent were men and 39% were NUC cases. Eightytwo percent had arterial hypertension, 66% obesity and 46% diabetes. Of those, 49% received coumarin anticoagulants. All patients with NUC and 75% with UC presented ulcers with necrosis, located more frequently on the legs. In 72% of the cases the histological diagnosis was made with one biopsy. In all the treatment was multimodal and mortality at one year was 42%. CONCLUSION: We observed a high proportion of patients with NUC, in relation to what is reported in the literature, and that half received vitamin K antagonists. The histological diagnosis was made with one biopsy in most of the cases, as the surgical technique for taking the sample, the Von Kossa staining and the evaluation by an expert pathologist were the key of it.

Introducción: La calcifilaxis es un trastorno vascular grave caracterizado por depósito de calcio en túnica media arteriolar, trombosis y necrosis cutánea. Se describió en pacientes con insuficiencia renal (CU), aunque puede producirse en su ausencia (CNU). Sus factores de riesgo están en estudio y su diagnóstico puede ser complejo. Su mortalidad se estimaba en 60-80%, aunque trabajos recientes indican que ha disminuido (40%). Métodos: Estudio retrospectivo entre el 1/1/2011 y el 31/12/2019. Se revisaron los antecedentes, las características clínicas, los hallazgos de laboratorio e histopatológicos, y la evolución de todos los pacientes con diagnóstico de calcifilaxis evaluados en el Hospital Italiano de Buenos Aires. Resultados: Se incluyeron 39 pacientes. El 61.5% (24) eran hombres y 38.5% (15) fueron casos de CNU. De éstos, 82% presentaba hipertensión arterial, 66% obesidad y 46% diabetes. El 49% recibía anticoagulantes dicumarínicos. Todos los pacientes con CNU y 75% con CU presentaron úlceras de fondo necrótico, localizadas con mayor frecuencia en las piernas. En 72% de los casos el diagnóstico histológico se efectuó con una toma de biopsia. En todos, el tratamiento fue multimodal y la mortalidad al año fue de 42%. Conclusión: Observamos una elevada proporción de pacientes con CNU, en relación con lo comunicado en la literatura, y la mitad recibía anticoagulantes dicumarínicos. El diagnóstico histológico se efectuó por biopsia en la mayor parte de los casos, para lo cual la toma quirúrgica de la muestra, la tinción con Von Kossa y la evaluación por un patólogo experto fueron claves.

The Australian Calciphylaxis Registry: reporting clinical features and outcomes of patients with calciphylaxis.

BACKGROUND: Calciphylaxis is a rare disease, predominantly affecting patients with chronic kidney disease (CKD) and associated with significant morbidity and mortality due to progressive cutaneous calcification, necrotic ulceration and infection. Clinical registries have been established to better understand the risk factors, optimal treatments and disease outcomes of calciphylaxis. METHODS: We established a prospective, Internet-based clinical registry for the online notification of calciphylaxis cases in Australia. Seven institutions participated, with data recorded on patient characteristics, biochemical parameters, treatments and disease outcomes. RESULTS: Between 2014 and 2019, 47 cases of calciphylaxis were registered. The mean patient age was 66 ± 11 years and body mass index was 35 ± 9 kg/m2, with a higher proportion of females (51%). Eighty-seven percent of patients had end-stage kidney disease (ESKD), with 61% on hemodialysis or hemodiafiltration, with a median dialysis vintage of 4.8 [interquartile range (IQR) 1.7-7.4)] years. Five patients had CKD not requiring dialysis and two were kidney transplant recipients. Diabetes was present in 76% of patients and the cause of ESKD in 60%; 34% received vitamin K antagonists (VKAs) before diagnosis. The median parathyroid hormone level at diagnosis was 32 (IQR 14-50) pmol/L. The most common site of calciphylaxis was the lower limbs (63%), with 19% of patients having more than one area involved. Ten patients (22%) had a resolution of calciphylaxis and 25 died, with 50% mortality at a median of 1.6 (IQR 0.2-2.5) years from diagnosis. CONCLUSIONS: The Australian Calciphylaxis Registry highlights risk factors for calciphylaxis, including diabetes, obesity and VKA use. Resolution of calciphylaxis is uncommon despite multimodal therapy and mortality from calciphylaxis in the first year following diagnosis remains high.

Calcifilaxis / Calciphylaxis

Resumen La calcifilaxis es una enfermedad poco frecuente, aunque presenta una elevada tasa de mortalidad debido sobre todo a complicaciones como sepsis o gangrena. Generalmente se asocia a insuficiencia renal severa. Se define como la calcificación de la capa media de vasos de pequeño y mediano tamaño de la dermis y tejido celular subcutáneo. Clínicamente se manifiesta como un síndrome de livedoracemosa que progresa a púrpura retiforme y necrosis cutánea. La primera línea de tratamiento es el tiosulfato sódico.

Abstract Calciphylaxis is a rare disease, although it has a high mortality rate due mainly to complications such as sepsis or gangrene. It is usually associated with severe renal failure. It is defined as the calcification of the middle layer of small and medium-sized vessels of the dermis and subcutaneous cellular tissue. Clinically it manifests as a livedoracemosa syndrome that progresses to retinal purpura and cutaneous necrosis. The first line of treatment is sodium thiosulfate.

Calciphylaxis epidemiology, risk factors, treatment and survival among French chronic kidney disease patients: a case-control study.

BACKGROUND: Calcific Uremic Arteriolopathy (CUA) is a rare disease, causing painful skin ulcers in patients with end stage renal disease. Recommendations for CUA management and treatment are lacking. METHODS: We conducted a retrospective cohort study on CUA cases identified in western France, in order to describe its management and outcome in average clinical practices. Selection was based on the Hayashi diagnosis criteria (2013) extended to patients with eGFR < 30 mL/min/1.73m2. Dialyzed CUA cases were compared with 2 controls, matched for age, gender, region of treatment and time period. RESULTS: Eighty-nine CUA cases were identified between 2006 and 2016, including 19 non dialyzed and 70 dialyzed patients. Females with obesity (55.1%) were predominant. Bone mineral disease abnormalities, inflammation and malnutrition (weight loss, serum albumin decrease) preceded CUA onset for 6 months. The multimodal treatment strategy included wound care (98.9%), antibiotherapy (77.5%), discontinuation of Vitamin K antagonists (VKA) (70.8%) and intravenous sodium thiosulfate (65.2%). 40.4% of the patients died within the year after lesion onset, mainly under palliative care. Surgical debridement, distal CUA, localization to the lower limbs and non calcium-based phosphate binders were associated with better survival. Risks factors of developing CUA among dialysis patients were obesity, VKA, weight loss, serum albumin decrease or high serum phosphate in the 6 months before lesion onset. CONCLUSION: CUA involved mainly obese patients under VKA. Malnutrition and inflammation preceded the onset of skin lesions and could be warning signs among dialysis patients at risk. TRIAL REGISTRATION: ClinicalTrials.gov identifier NCT02854046, registered August 3, 2016.

Calciphylaxis in Patients With Normal Renal Function: A Case Series and Systematic Review.

OBJECTIVE: To define concomitant risk factors, treatment, and outcomes for patients with nonnephrogenic calciphylaxis (NNC). PATIENTS AND METHODS: A retrospective review of Massachusetts General Hospital (MGH) medical records (January 1, 2014, through February 29, 2016) and a systematic literature review of PubMed, Google Scholar, EMBASE, MEDLINE, and CENTRAL (August 1, 1970, through July 31, 2016) were performed. Demographic characteristics and concomitant features were summarized and compared between patients with different lesion characteristics. Outcomes (lesion improvement and mortality) and their predictors were analyzed. RESULTS: Nine patients (median age, 72 years [interquartile range (IQR), 44-82 years]; 78% women; 89% white race) were identified through MGH records. The literature review identified 107 patients (median age, 60 years [IQR, 49-72 years]; 77% women; 86% white race). Vitamin K antagonism and obesity were the most common concomitant factors. In the literature review, lower age (P<.001) and higher body mass index (P=.03) were associated with the central location of lesions, whereas vitamin K antagonism was associated with the peripheral location (P=.009). In the MGH series, median survival was 24.0 months (95% CI, 7.8-36.0 months), and 33% (95% CI, 14%-60%) had lesion improvement by 6 months. In the literature review, median survival was 4.2 months (95% CI, 1.9-5.9 months), median time to lesion improvement was 5.9 months (95% CI, 3.9-8.9 months), and none of the treatments were associated with lesion improvement or survival. CONCLUSION: This description of concomitant traits may augment an earlier recognition of NNC. Future research is needed to investigate NNC pathogenesis and treatments.

Clinical characteristics and risk factors associated with mortality in calcific uremic arteriolopathy.

BACKGROUND: Calcific uremic arteriolopathy (CUA) is an often-fatal condition in dialysis patients. The clinical descriptions and treatments of CUA patients have been confined mostly to case reports. We report a comprehensive characterization of CUA and its associated diagnosis, treatment patterns, and outcome. METHODS: An internet-based registry collected information about CUA in dialysis patients. Univariate analysis using Cox proportional hazards models estimated hazard ratios of the association between clinical characteristics, laboratory values, and treatments with all-cause mortality. RESULTS: A total of 117 CUA patients had adequate information for analysis. The majority of patients (56.7%) were diagnosed clinically, with only 32.5% biopsied. Debridement was undertaken in 42.6% of cases. Intravenous sodium thiosulfate (STS) was initiated in 54.7% of patients; most received ≥ 12.5 g of STS (98.3%) for < 3 months (79.7%). Mean parathyroid hormone (PTH) and phosphorus (P) were 459 ± 492 pg/mL and 6.3 ± 2.1 mg/dL, respectively. A total of 24 patients (21.6%, of 111 with information) died, with a median survival time of 2.9 months. In univariate analysis, higher mortality was observed in patients with cardiovascular disease (CVD; HR = 10.47; 95% CI 1.40-78.38), those taking warfarin at time of diagnosis (HR = 2.74; 95% CI 1.16-6.51), and those who had both diabetes (DM) and CVD and who were taking warfarin (HR = 13.41; 95% CI 1.66-109.29). CONCLUSIONS: In real-world clinical practice, there is substantial variability in the diagnosis and treatment of CUA. There is usually only modest derangement of bone and mineral parameters at the time of diagnosis. Death is common. The presence of CVD and use of warfarin may influence clinical outcome after diagnosis of CUA.

Management of a Complex Peristomal Calciphylaxis: A Case Study.

BACKGROUND: Calciphylaxis, also referred to as calcific uremic arteriolopathy, is a rare and serious syndrome of small blood vessels with a high mortality rate. Calciphylaxis lesions require intensive wound management with medical interventions for the patient to survive the sequelae of sepsis and present unique challenges when found in the peristomal skin. CASE: A 33-year-old man presented with multiple malodorous stage 4 pressure injuries of his sacrococcygeal and gluteal area. His medical history included chronic kidney disease requiring hemodialysis since 2007, diabetes mellitus, and incomplete paraplegia. He underwent diverting colostomy to enhance wound healing. His hospital stay was complicated by the development of a peristomal calciphylaxis lesion (PCL) that made ostomy pouching especially challenging for the nursing staff. His care needs were also aggravated by nonadherence to diet restriction, pressure injury prevention efforts, and a continued need for high doses of analgesic medication. Collectively, these issues presented a challenge for the health care team during his hospital course and during safe discharge planning. CONCLUSIONS: The peristomal calciphylaxis lesion decreased in surface area and improved in appearance with the use of various wound care products as his medical condition improved. Skilled nursing management in the context of ongoing interdisciplinary collaboration assisted in managing the patient's peristomal calciphylaxis, ultimately leading to safe discharge from hospital.

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.

Calciphylaxis: A Disease of Pannicular Thrombosis.

OBJECTIVE: To identify coagulation risk factors in patients with calciphylaxis and the relationship between anticoagulation use and overall survival. PATIENTS AND METHODS: Study subjects were 101 patients with calciphylaxis seen at Mayo Clinic from 1999 to September 2014. Data including thrombophilia profiles were extracted from the medical records of each patient. Survival status was determined using patient registration data and the Social Security Death Index. Survival was estimated using the Kaplan-Meier method, and associations were evaluated using Cox proportional hazards models. RESULTS: Sixty-four of the 101 patients underwent thrombophilia testing. Of these, a complete test panel was performed in 55 and a partial panel in 9. Severe thrombophilias observed in 60% (33 of 55) of the patients included antiphospholipid antibody syndrome protein C, protein S, or antithrombin deficiencies or combined thrombophilias. Of the 55 patients, severe thrombophilia (85%, 23 of 27) was noted in patients who were not on warfarin at the time of testing (27). Nonsevere thrombophilias included heterozygous factor V Leiden (n=2) and plasminogen deficiency (n=1). For the comparison of survival, patients were divided into 3 treatment categories: Warfarin (n=63), other anticoagulants (n=20), and no anticoagulants (n=18). There was no statistically significant survival difference between treatment groups. CONCLUSION: Laboratory testing reveals a strikingly high prevalence of severe thrombophilias in patients with calciphylaxis, underscoring the importance of congenital and acquired thrombotic propensity potentially contributing to the pathogenesis of this disease. These findings may have therapeutic implications; however, to date, survival differences did not vary by therapeutic choice.

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.

Sodium thiosulfate therapy for calcific uremic arteriolopathy.

BACKGROUND AND OBJECTIVE: Calcific uremic arteriolopathy (CUA) is an often fatal condition with no effective treatment. Multiple case reports and case series have described intravenous sodium thiosulfate (STS) administration in CUA, but no studies have systematically evaluated this treatment. DESIGN, SETTING, PARTICIPANTS, & MEASUREMENTS: This study included 172 patients undergoing maintenance hemodialysis who had CUA and were treated with STS between August 2006 and June 2009 at Fresenius Medical Care North America. Of these, 85% completed STS therapy. Clinical, laboratory, and mortality data were abstracted from clinical information systems. Responses to survey questionnaires sent to treating physicians regarding patient-level outcomes were available for 53 patients. Effect on CUA lesions and mortality were summarized as CUA outcomes. Relevant laboratory measures, weight (using pairwise comparisons of values before, during, and after STS), and adverse events were summarized as safety parameters. RESULTS: Mean age of the cohort was 55 years, and 74% of patients were women. Median STS dose was 25 g, and median number of doses was 38. Among surveyed patients, CUA completely resolved in 26.4%, markedly improved in 18.9%, improved in 28.3%, and did not improve in 5.7%; in the remaining patients (20.8%), the response was unknown. One-year mortality in patients treated with STS was 35%. Adverse events, laboratory abnormalities, and weight-related changes were mild. Significant reductions in serum phosphorous (P=0.02) and parathyroid hormone (P=0.01) were noted during STS treatment in patients who completed the therapy. CONCLUSIONS: Although conclusive evidence regarding its efficacy is lacking, a majority of patients who received STS demonstrated clinical improvement in this study.

Use of sodium thiosulphate in a multi-interventional setting for the treatment of calciphylaxis in dialysis patients.

BACKGROUND: Calciphylaxis is a life-threatening complication in patients with end-stage renal disease (ESRD). No established therapy exists so far. The aim of the present study was to determine the therapeutic response to a multi-interventional treatment regimen with consistent use of sodium thiosulphate (STS) in an Austrian cohort of calciphylaxis patients. METHODS: We retrospectively collected demographic, clinical and laboratory data on 27 calciphylaxis patients treated with STS at seven Austrian dialysis centres between June 2004 and November 2010. RESULTS: Twenty-seven dialysis patients (68 ± 12 years) were treated with STS for a median (25th, 75th percentile) of 96 (54, 133) days. Seven patients (26%) suffered from proximal-type, and 20 patients (74%) from distal-type calciphylaxis. Fourteen patients (52%) showed a complete remission, five patients (19%) a partial remission and eight patients (30%) progression that resulted in amputation in four patients. During a median follow-up of 101 (79, 273) days, 14 patients died (52%). Non-survivors were older (P = 0.04), showed higher CRP values (P = 0.04), presented more frequently with proximal-type calciphylaxis (P = 0.03), had a higher disease severity score at diagnosis (P = 0.01), were treated more often with antibiotics (P = 0.01) and cinacalcet (P = 0.03) and had a lower remission rate during treatment (P = 0.004) than did survivors. The use of antibiotics and cinacalcet, disease severity at diagnosis and remission rates were found to be significant survival predictors in logistic regression analysis. CONCLUSIONS: Calciphylaxis remains a serious complication with high mortality. Early and consistent therapy including STS may help to improve the disease outcome.

[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.

[Sodium thiosulfate--new hope for the treatment of calciphylaxis]. / Natriumtiosulfaatti --toivoa kalsifylaksian hoitoon.

Calciphylaxis is a rare disease primarily affecting patients dependent on dialysis. It is characterised by small vessel media calcification leading to cutaneous ischemia and necrosis. The mortality rate is high with infection and sepsis being the most common causes of death. Calcium salts, vitamin D and high levels of serum calcium and phosphorus increase the risk of calciphylaxis. Current therapies including restoration of mineral homeostasis, wound care and pain control, are not entirely effective. Sodium thiosulfate, by dissolving calcium deposits, is a novel therapeutic choice for calciphylaxis. It has proved successful also in cases refractory to conventional treatment.

Efficacy of sodium thiosulfate for the treatment for calciphylaxis.

BACKGROUND/AIMS: Calciphylaxis is a rare condition with dismal prognosis that affects patients with ESRD. Sodium thiosulfate (STS) may play a therapeutic role but its proposed efficacy is based on case reports and thus subject to publication bias. METHODS: We identified all patients who received STS for any indication over a 5-year period through pharmacy records of 4 hospitals, retrospectively reviewing medical records for risk factors, laboratory values, the response of skin lesions to STS, and mortality. RESULTS: 14 patients received STS for calciphylaxis over 5 years. Following STS administration, pain decreased in 71% of patients, and 70% had an improvement in their lesions. Those who did not improve or stabilize their skin lesions tended to have more advanced skin lesions, were on renal replacement therapy longer, were more obese and received less total dose of STS. However, despite STS, there was a 71% mortality rate, with 50% of subjects dying within 6 months. CONCLUSION: We conclude in this study of all subjects who received STS at our Institution that STS is an effective treatment for the pain and skin lesions of calciphylaxis if given in the early stages of disease and for a consistent period of time. However, there is little impact on overall mortality compared to historical published cohorts.

Sodium thiosulfate-based treatment in calcific uremic arteriolopathy: a consecutive case series.

BACKGROUND: Calcific uremic arteriolopathy (CUA) is a rare complication in end stage renal disease with high mortality. Numerous case reports and one case series of 3 patients report the benefit of sodium thiosulfate (STS) for treatment of CUA. The purpose of this evaluation was to examine the response to a STS-based treatment approach in patients with CUA with 1 year follow up. METHODS: A retrospective case series of 6 consecutive patients from Manitoba, Canada who met predefined diagnostic criteria for CUA and received STS between 2006 and 2008 were included. STS responders were defined as improvement in at least one of the following three parameters: pain severity, wound size and diagnostic imaging/radiography. Mortality, STS dose, duration, adverse events and cost were also collected. RESULTS: Four patients were classified as responders. The 2 responders who survived at 1 year of follow-up demonstrated an improvement in all 3 parameters examined including an improvement in their follow-up diagnostic imaging results within the first 4 - 6 weeks of STS treatment. At 1 year of follow-up, 3 patients died. CONCLUSION: Using an STS-based multifaceted treatment approach for CUA, 4 patients responded but 3 of 6 patients died within 1 year. Further larger prospective studies are needed to delineate STS responders from non-responders.

Determinants of survival in patients with calciphylaxis: a multivariate analysis.

BACKGROUND: Our study aims to assess the factors affecting survival in patients with calciphylaxis. METHODS: We identified 26 patients with biopsy-proven calciphylaxis treated between 1995 and 2007. Clinical and follow-up data were obtained from medical records. Cox proportional hazards models were used to assess the factors affecting survival. RESULTS: The study group consisted of 23 women and 3 men with a mean age of 56.4 +/- 12.9 years. All patients had multiple comorbidities/risk factors including coronary artery disease (58%), diabetes mellitus (58%), and peripheral vascular disease (23%). Mean laboratory values were: calcium, 9.0 mg/dL (range, 6.8-11.6); albumin, 2.8 mg/dL; phosphate, 4.5 mg/dL (range, 2.5-7.5); Ca *phosphate, 35.9; and parathyroid hormone, 320.9 pg/mL (range, 4.6-2,419). Parathyroidectomy was performed in 9 of 26 patients (35%). Of our patients, 19% underwent revascularization procedures and 58% underwent debridement. In multivariate analyses, factors associated with poor survival were female gender ( P = .01), increased weight ( P = .01), and need for vascular procedures ( P = .06). Improved survival was associated with operative debridement ( P = .01). Parathyroidectomy alone did not emerge as a determinant of patient survival, although there was a trend to improved survival when debridement and parathyroidectomy were combined ( P = .09). CONCLUSION: Rather than a single intervention such as parathyroidectomy, a multidisciplinary approach involving early diagnosis, aggressive medical management, operative debridement, and parathyroidectomy may improve survival in calciphylaxis.