title: Lack of evidence does not justify neglect: how can we address unmet medical needs in calciphylaxis?
abstract: Calcific uraemic arteriolopathy (CUA), or calciphylaxis, is a rare disease predominantly occurring in comorbidity with dialysis. Due to the very low frequency of CUA, prospective studies on its management are lacking and even anecdotal reports on treatment remain scarce. Therefore, calciphylaxis is still a challenging disease with dismal prognosis urgently requiring adequate strategies for diagnosis and treatment.In an attempt to fill some of the current gaps in evidence on various, highly debated and controversial aspects of dialysis-associated calciphylaxis, 13 international experts joined the 1st Consensus Conference on CUA, held in Leuven, Belgium on 21 September 2015. The conference was supported by the European Calciphylaxis Network (EuCalNet), which is a task force of the ERA-EDTA scientific working group on Chronic Kidney Disease-Mineral and Bone Disorders (CKD-MBD). After an intense discussion, a 9-point Likert scale questionnaire regarding 20 items on calciphylaxis was anonymously answered by each participant. These 20 items addressed unsolved issues in terms of diagnosis and management of calciphylaxis. On the one hand, the analysis of the expert opinions identified areas of general consensus, which might be a valuable aid for physicians treating such a disease with less experience in the field. On the other hand, some topics such as the pertinence of skin biopsy and administration of certain treatments revealed divergent opinions. The aim of the present summary report is to provide some guidance for clinicians who face patients with calciphylaxis in the current setting of absence of evidence-based medicine.
authors.affiliation: Department of Cardiology, University Hospital RWTH Aachen, Aachen, Germany.
authors.forename: Vincent M
authors.initials: VM
authors.lastname: Brandenburg
authors.affiliation: Department of Internal Medicine, Division of Nephrology, University Hospitals Leuven, Leuven, Belgium.
authors.forename: Pieter
authors.initials: P
authors.lastname: Evenepoel
authors.affiliation: Department of Nephrology, RWTH University of Aachen, Aachen, Germany.
authors.forename: Jürgen
authors.initials: J
authors.lastname: Floege
authors.affiliation: Renal and Transplantation Department, Guy's and St Thomas' Hospitals, London, UK.
authors.forename: David
authors.initials: D
authors.lastname: Goldsmith
authors.affiliation: Department of Nephrology, RWTH University of Aachen, Aachen, Germany.
authors.forename: Rafael
authors.initials: R
authors.lastname: Kramann
authors.affiliation: Division of Nephrology, Ambroise Paré University Hospital, APHP, University of Paris Ouest-Versailles-St-Quentin-en-Yvelines (UVSQ), Boulogne-Billancourt, Paris INSERM U1018, Research Centre in Epidemiology and Population Health (CESP) Team 5, Villejuif, France F-CRIN-INI-CRCT, Nancy, France.
authors.forename: Ziad
authors.initials: Z
authors.lastname: Massy
authors.affiliation: Department of Cardiovascular, Respiratory, Nephrologic, Geriatric Sciences, Sapienza University of Rome, Rome, Italy.
authors.forename: Sandro
authors.initials: S
authors.lastname: Mazzaferro
authors.affiliation: Department of Biochemistry, Faculty of Medicine, Health and Life Science, Maastricht, The Netherlands.
authors.forename: Leon J
authors.initials: LJ
authors.lastname: Schurgers
authors.affiliation: Department of Renal Medicine, Manchester Academic Health Sciences Centre, Salford Royal NHS Foundation Trust, Salford, UK.
authors.forename: Smeeta
authors.initials: S
authors.lastname: Sinha
authors.affiliation: Nephrology and Renal Transplantation Department, Hospital Clinic, University of Barcelona, Barcelona, Spain.
authors.forename: Vicens
authors.initials: V
authors.lastname: Torregrosa
authors.affiliation: Department of Nephrology and Dialysis, Clinique du Landy, Saint-Ouen, France Department of Renal Physiology, Necker Hospital, University of Paris Descartes, Paris, France.
authors.forename: Pablo
authors.initials: P
authors.lastname: Ureña-Torres
authors.affiliation: Department of Nephrology, VU University Medical Center, Amsterdam, The Netherlands.
authors.forename: Marc
authors.initials: M
authors.lastname: Vervloet
authors.affiliation: Department of Health Sciences, University of Milan, Renal Division, San Paolo Hospital, Milan, Italy.
authors.forename: Mario
authors.initials: M
authors.lastname: Cozzolino
mesh_terms: D002115:Calciphylaxis / Q000473:pathology* / Q000517:prevention & control*; D019468:Disease Management; D019317:Evidence-Based Medicine; D006801:Humans; D020380:Needs Assessment
keywords: CKD-MBD; calciphylaxis; cardiovascular; chronic renal failure; mineral metabolism