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In advanced chronic kidney disease (ACKD), the phospho-calcium imbalance present is directly related to the development of lesions. However, in the case of non-uremic calciphylaxis (NUC), only several risk factors have been identified, choosing to extrapolate the treatment of uremic calciphylaxis (UC)<a class="elsevierStyleCrossRefs" href="#bib0005"><span class="elsevierStyleSup">1,2</span></a>. While UC and NUC are known for high mortality rates (up to 52%), a number of recent NUC cases have been published with higher survival rates<a class="elsevierStyleCrossRefs" href="#bib0010"><span class="elsevierStyleSup">2–5</span></a>. In this paper we report our experience with 12 cases.</p><p id="par0010" class="elsevierStylePara elsevierViewall">A retrospective study of cases diagnosed with NUC at the General Hospital of Granollers, between April 2013 and December 2021, was conducted. The inclusion criteria were patients with biopsied lesions with a diagnosis of calciphylaxis. Patients with ACKD were excluded, as well as those with loss to follow-up. Demographic data, risk factors described in the literature and progression under treatment with sodium thiosulfate (25 mg intravenous/3 times per week)<a class="elsevierStyleCrossRefs" href="#bib0005"><span class="elsevierStyleSup">1–5</span></a> were collected.</p><p id="par0015" class="elsevierStylePara elsevierViewall">Fifteen cases were identified in the period described. Three were discarded due to loss to follow-up. Of the 12 patients included, all were Caucasian, 66.7% (<span class="elsevierStyleItalic">n</span> = 8) were female, with a median age of 78.4 years (IQR: 71.6-85 years). 66.7% (<span class="elsevierStyleItalic">n</span> = 8) were obese (BMI: 34.2 kg/m<span class="elsevierStyleSup">2</span> [IQR: 24.2–40.2 kg/m<span class="elsevierStyleSup">2</span>]). The median glomerular filtration rate was 53.5 ml/min/1.73 m<span class="elsevierStyleSup">2</span> (IQR: 37.5–62.5 ml/min/1.73 m<span class="elsevierStyleSup">2</span>). All were multi-pathological, 100% (<span class="elsevierStyleItalic">n</span> = 12) hypertensive and 66.7% (<span class="elsevierStyleItalic">n</span> = 8) were type 2 diabetic. 33.3% (<span class="elsevierStyleItalic">n</span> = 4) had a systemic disease (one psoriatic arthritis, 2 rheumatoid arthritis, one lupus), none of them had liver disease or secondary hyperparathyroidism. 8.3% (<span class="elsevierStyleItalic">n</span> = 1) had osteoporosis. None had coagulopathies, and only 8.3% (<span class="elsevierStyleItalic">n</span> = 1) were, diagnosed with tumour disease (prostate adenocarcinoma).</p><p id="par0020" class="elsevierStylePara elsevierViewall">No abnormalities in calcaemia, phosphatemia or alkaline phosphatase were detected. 50% (<span class="elsevierStyleItalic">n</span> = 6) had malnutrition parameters (median albumin of 3.4 g/dl (IQR: 2.8–3.9 g/dl). Regarding concomitant treatments; 50% (<span class="elsevierStyleItalic">n</span> = 6) were following treatment with acenocoumarol, 41.7% (<span class="elsevierStyleItalic">n</span> = 5) chronic corticotherapy, 83.3% (<span class="elsevierStyleItalic">n</span> = 12) vitamin D supplementation and 16.7% (<span class="elsevierStyleItalic">n</span> = 2) oral iron. All had lesions in the lower limbs. Some 16.7% (<span class="elsevierStyleItalic">n</span> = 2) required dose adjustment, with metabolic acidosis being the most common side effect (83.3%). The anticoagulant drug was changed for all those previously treated with acenocoumarol. Some 58.3% (<span class="elsevierStyleItalic">n</span> = 7) had complete resolution (<a class="elsevierStyleCrossRef" href="#fig0005">Fig. 1</a>), 25% (<span class="elsevierStyleItalic">n</span> = 3) lesion improvement, 8.3% (<span class="elsevierStyleItalic">n</span> = 1) experienced stability, and only 8.3% (<span class="elsevierStyleItalic">n</span> = 1) experienced a clinical deterioration of the lesions. The median treatment duration was 20.7 weeks (IQR: 18-22.7 weeks). Total mortality was 41.7% (<span class="elsevierStyleItalic">n</span> = 5), 2 related to NUC (septic shock) and 3 others secondary to decompensation of the underlying disease.</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><p id="par0025" class="elsevierStylePara elsevierViewall">Among our results, we highlight the fact that all patients had more than one concomitant factor described for NUC<a class="elsevierStyleCrossRefs" href="#bib0005"><span class="elsevierStyleSup">1–5</span></a>, in addition to being multi-pathological; raising suspicion of a multifactorial aetiology. Although Bajaj et al.<a class="elsevierStyleCrossRef" href="#bib0025"><span class="elsevierStyleSup">5</span></a> highlight the relationship between the high prevalence of patients with vitamin K antagonists, the fact that 57.1% of our patients were not treated with these drugs casts doubt on whether this is a determining factor in the pathogenesis. All had lower limb ulcers, suggesting a probable anatomical predisposing factor, such as venous return.</p><p id="par0030" class="elsevierStylePara elsevierViewall">Despite the fact that UC involves a phospho-calcium imbalance, all our subjects showed normality in these parameters. This is why we believe that its determination should not constitute a diagnostic mainstay. We highlight our positive experience with sodium thiosulfate, observing that most patients obtained complete resolution, along with lesion healing. It is important to highlight that the cure rate is biased by the high mortality rate since, out of 5 deaths, only 2 of them were directly related to NUC, the rest were linked to the decompensation of underlying diseases.</p><p id="par0035" class="elsevierStylePara elsevierViewall">In conclusion, NUC is a poorly studied disease with few articles clarifying its pathophysiology. Treatment with sodium thiosulfate is effective. We emphasize the need to maintain a multidisciplinary approach, considering the comorbidities that occur in conjunction with this disease.</p><span id="sec0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0005">Ethical responsibilities</span><p id="par0040" class="elsevierStylePara elsevierViewall">The authors confirm that they have obtained the informed consent of the patients and have complied with the ethical standards relevant to the conduct of this study. This work has been consulted and approved by the Clinical Research Ethics Committee of the Hospital General de Granollers.</p></span><span id="sec0010" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0010">Funding</span><p id="par0045" class="elsevierStylePara elsevierViewall">The current study has not required funding for its completion.</p></span></span>" "textoCompletoSecciones" => array:1 [ "secciones" => array:3 [ 0 => array:2 [ "identificador" => "sec0005" "titulo" => "Ethical responsibilities" ] 1 => array:2 [ "identificador" => "sec0010" "titulo" => "Funding" ] 2 => array:1 [ "titulo" => "References" ] ] ] "pdfFichero" => "main.pdf" "tienePdf" => true "multimedia" => array:1 [ 0 => array:8 [ "identificador" => "fig0005" "etiqueta" => "Figure 1" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr1.jpeg" "Alto" => 433 "Ancho" => 805 "Tamanyo" => 48566 ] ] "detalles" => array:1 [ 0 => array:3 [ "identificador" => "at0005" "detalle" => "Figure " "rol" => "short" ] ] "descripcion" => array:1 [ "en" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">Resolution of a non-uremic calciphylaxis lesion after completion of treatment with intravenous sodium thiosulfate.</p>" ] ] ] "bibliografia" => array:2 [ "titulo" => "References" "seccion" => array:1 [ 0 => array:2 [ "identificador" => "bibs0005" "bibliografiaReferencia" => array:5 [ 0 => array:3 [ "identificador" => "bib0005" "etiqueta" => "1" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Calciphylaxis from nonuremic causes: A systematic review" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:4 [ 0 => "S.U. 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Journal Information
Vol. 160. Issue 7.
Pages 318-319 (April 2023)
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Vol. 160. Issue 7.
Pages 318-319 (April 2023)
Scientific letter
Non-uremic calciphylaxis: Case series
Calcifilaxis no urémica: descripción de una serie de casos
Carlos César Martí Pelluch
, Anna Soler Sendra, Mònica Vila Vall-llovera
Corresponding author
Servicio de Medicina Interna, Hospital General de Granollers, Granollers, Barcelona, Spain
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From Monday to Friday from 9 a.m. to 6 p.m. (GMT + 1) except for the months of July and August which will be from 9 a.m. to 3 p.m.
Calls from Spain
932 415 960
Calls from outside Spain
+34 932 415 960
E-mail