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Vol. 27. Issue. 5.October 2007
Pages 527-659
Vol. 27. Issue. 5.October 2007
Pages 527-659
DOI:
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Calcific uraemic arteriolopathy (calciphylaxis): incidence, clinical features and long term outcomes
ARTERIOLOPATÍA URÉMICA CALCIFICANTE (CALCIFILAXIS): INCIDENCIA, FORMAS DE PRESENTACIÓN Y EVOLUCIÓN.
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Vicent Esteve Simó, Jaume Almirall, Jesus Luelmo*, Amparo Sáez**, Xavier Andreu**, Manuel García
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CALCIFIC URAEMIC ARTERIOLOPATHY (CALCIPHYLAXIS): INCIDENCE, CLINICAL FEATURES AND LONG TERM OUTCOMES. Calcific uraemic arteriolopathy, also named calciphylaxis, is a rare but serious disorder characterized by medial mural calcification of small vessel leading to tissue ischaemia. It most commonly occurs in end stage renal disease patients on dialysis or recently received renal trasplant with chronic nephropathy allograft. The pathogenesis of calciphylaxis is poorly understood. Abnormalities in mineral metabolism are clearly involved, but the specific factors that induces this disorder are not completely known. Objectives: Describe the main clinical features, outcomes and follow up of all calciphylaxis cases recorded in our dialysis unit in order to analise the incidence, the main biologic parameters and the therapeutic background in wich calciphylaxis appeared. Material and methods: We performed a descriptive study about all the calciphylaxis cases diagnosed at our dialysis unit beetwen the years 1991 and 2005. Results: 8 cases, 6 women. Mean age: 65.3 years. All the patients were on haemodialysis treatment (one previous renal transplant). Mean time on dialysis was 76.6 months. Cumulative incidence was 1.17%. The principal end stage renal disease aethiology was neprhoangioeslerosis in four patients. Secondary hiperparatyrhoidism was present in 4 patients and 2 of them had been paratyrhoidectomized previously. A second cutaneus biopsy was needed for correct diagnosis in 3 patients. Calciphylaxis distal lesions were present in 7 patients. Two cases required urgent paratyrhoidectomy in order to control calciphylaxis. Only in 2 cases a Ca x P product > 60 mg/dL was present and 3 cases had PTHi values higher than 300 pg/mL. Calcium phospate binders and vitamin D were present in 2 and 4 cases, respectively. One patient with proximal calciphylaxis died due to skin injury infection. Conclusions: Calciphylaxis is a rare disorder but not exceptional, related to end stage renal disease patients. The diagnosis requires a high clinical suspicion, beeing sometimes dificult to distinguish from other entities in spite of pathological study. Proximal distribution of calciphylaxis had worst prognostic. Metabolic disorders and therapeutics background were not different from other patients included in dialysis treatment.
Keywords:
Calciphylaxis, renal disease, dialysis
La arteriolopatía urémica calcificante, también conocida como calcifilaxis, es una entidad caracterizada por la presencia de áreas de necrosis isquémica junto con extensas calcificaciones de la capa media de las arteriolas dermoepidérmicas. Fundamentalmente se desarrolla en pacientes con insuficiencia renal en diálisis o trasplantados con disfunción del injerto. Aunque las alteraciones propias del estado urémico y del metabolismo calcio-fósforo son importantes, su etiopatogenia es compleja; siendo los mecanismos desencadenantes poco conocidos. Objetivos: Describir las formas de presentación y evolución de los casos de calcifilaxis diagnosticados en nuestra unidad de diálisis. Calcular la incidencia y analizar el contexto biológico y terapéutico previo al episodio de calcifilaxis. Material y métodos: Análisis descriptivo de todos los casos de calcifilaxis diagnosticados en nuestra unidad durante el período comprendido entre 1991-2005. Resultados: 8 casos, 6 mujeres. Edad media: 65,3 años. Todos los pacientes incluidos en programa de Hemodiálisis (1 trasplante renal previo). Tiempo medio en diálisis de 76,6 meses. La incidencia acumulada fue de 1,17%. La principal etiología de la IRC fue la Nefroangioesclerosis (4 pacientes). Existía antecedentes de hiperparatiroidismo secundario en 4 pacientes, con paratiroidectomía previa en 2 de ellos. En 3 pacientes el diagnóstico requirió una segunda biopsia cutánea. La distribución de las lesiones fue distal en 7 casos. En 2 casos se practicó paratiroidectomía urgente para el control de las lesiones. Tan sólo 2 casos presentaban producto Calcio-fósforo > 60 mg/dL y 3 casos cifras de PTHi > 300 pg/mL. 2 casos tomaban quelantes cálcicos y 4 suplementos de vitamina D. Un paciente con distribución proximal de las lesiones fue exitus por sobreinfección de las mismas. Conclusiones: La calcifilaxis es un proceso infrecuente. El diagnóstico requiere de una alta sospecha clínica, siendo en ocasiones difícil de distinguir de otros procesos. La localización a nivel proximal confiriere un peor pronóstico a las lesiones. Las alteraciones metabólicas y conductas terapéuticas son indistinguibles de las que presentan el resto de pacientes sometidos a diálisis.
Palabras clave:
Calcifilaxis, insuficiencia renal, diálisis
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