[Calciphylaxis: fatal complication of cardiometabolic syndrome in patients with end stage kidney disease]. / Calcifilaxis: complicación grave del síndrome cardio-metabólico en pacientes con enfermedad renal crónica terminal (ERCT).

UNLABELLED: Calciphylaxis characterized by schemic skin ulceration due to subcutaneous small arterioles calcification, is a rare disease but usually fatal. Disorders of calcium metabolism and vascular calcifications are common in dialysis patients but calciphylaxis prevalence is low in patients with end stage renal disease. So we proposed other emergent factors implicated in calciphylaxis development. METHODS: We studied retrospective 8 patients who developed calciphylaxis in our service from january 2001 to december 2006. RESULTS: All patients were female with mean age at diagnosis 68.5+/-6.7 years. All patients were receiving hemodialysis therapy and 6 patients had been receiving hemodialysis less than four months. Six patients had diabetes mellitus type II and all patients were obese (BMI >25 kg/m2). All patients had metabolic syndrome (APTIII) with bad control hypertension and 6 (75%) were receiving anticoagulation therapy with warfarin. Patients didn t have severe alterations of calcium metabolism, all had product calcium-phosphorus <55. All patients developed low blood pressure at the beginning of dialysis treatment (98.3+/-22.7/60+/-18,29 mmHg). 7 patients present proximal lesions in fatty regions like abdomen and thighs. Histopathologic examination reveals calcium deposits in arteriole-sized and small vessels with vascular thrombosis. Prognosis was poor, seven patients died secondary to a sepsis originated in infected cutaneous ulcers. CONCLUSIONS: calciphylaxis is a disease with poor prognosis and high mortality, without specific treatment actually. Female gender, obesity associated with diabetes mellitus and cardiometabolic syndrome, anticoagulant therapy with warfarin and low blood pressure associated with hemodialysis therapy, are risk factors to develop calciphylaxis, in absence of severe disorders of calcium metabolism. In these patients is important to avoid hypotension episodes during dialysis, dialysis hypotension appears to be an important risk factor who promotes ischemia of subcutaneous adipose tissue.

Calcifilaxis: complicación grave del síndrome cardio-metabólico en pacientes con enfermedad renal crónica terminal (ERCT) / Calciphylaxis: fatal complication of cardio-metabolic syndrome in patients with end stage kidney disease

La calcifilaxis, caracterizada por la ulceración isquémica de la piel secundaria a la calcificación de las pequeñas arteriolas subcutáneas, es una enfermedad poco frecuente pero con mal pronóstico. Los pacientes con ERCT tienen un riesgo alto de calcificaciones patológicas debido a las alteraciones del metabolismo calcio-fósforo, pero solo un pequeño número desarrollan esta enfermedad. Por ello es lógico pensar que hay otros factores que condicionan el desarrollo de la calcifilaxis. Métodos: Con el fin de identificar estos posibles factores implicados en su génesis, hemos analizado de forma retrospectiva las características de los 8 pacientes con ERCT que presentaron calcifilaxis en nuestro hospital entre de enero 2001 a diciembre 2006. Resultados: Los 8 pacientes eran mujeres con edad media de 68,5 ± 6,7 años. Todas presentaban ERCT en hemodiálisis periódica (HD) y en 6 casos la cacifilaxis apareció en los primeros 4 meses del inicio del tratamiento con HD. Seis de las pacientes eran diabéticas tipo 2 y todas eran obesas (IMC > 25 kg/m2), 3 con obesidad grado 4 o mórbida. Todas cumplían criterios de síndrome metabólico (APT III), habían sido hipertensas mal controladas y en un 75% de los casos recibían tratamiento con anticoagulantes cumarínicos por distintas causas. No presentaban alteraciones severas del metabolismo calcio-fósforo: todas tenían un producto CaxP < 55. En todos los casos se controló estrictamente la Presión arterial (PA) con el tratamiento con HD manteniendo cifras medias de PA de 98,3 ± 22,7/60 ± 18,29 mmHg en el momento de la aparición de los síntomas. La mayoría de las pacientes (7/8), presentaron las lesiones a nivel proximal en las zonas con mayor depósito graso como muslos y abdomen. El estudio histológico de las lesiones en todos los casos demostró calcificación de pequeñas arteriolas subcutáneas asociada a paniculitis y trombosis de pequeños vasos. La evolución clínica fue mala ya que siete de las ocho pacientes murieron como consecuencia de una sepsis de origen cutáneo. La exéresis quirúrgica de los nódulos no modificó la mala evolución. En conclusión: La paniculitis calcificante en pacientes con ERCT es una enfermedad rara pero de mal pronóstico y elevada mortalidad. El sexo mujer, la obesidad asociada a Diabetes y Síndrome metabólico, el tratamiento anticoagulante y el excesivo control de la presión arterial al inicio del tratamiento con HD pueden favorecer su aparición incluso en ausencia de alteraciones relevantes del metabolismo Ca-P-PTH. Debido a la epidemia actual de DM, obesidad y síndrome metabólico es de esperar que el número de pacientes con estas características que desarrollen ERCT y calcifilaxis vaya en aumento (AU)

Calciphylaxis characterized by schemic skin ulceration due to subcutaneous small arterioles calcification, is a rare disease but usually fatal. Disorders of calcium metabolism and vascular calcifications are common in dialysis patients but calciphylaxis prevalence is low in patients with end stage renal disease. So we proposed other emergent factors implicated in calciphylaxis development. Methods: We studied retrospective 8 patients who developed calciphylaxis in our service from january 2001 to december 2006. Results: All patients were female with mean age at diagnosis 68.5 ± 6.7 years. All patients were receiving hemodialysis therapy and 6 patients had been receiving hemodialysis less than four months. Six patients had diabetes mellitus type II and all patients were obese (BMI > 25 kg/m2). All patients had metabolic syndrome (APTIII) with bad control hypertension and 6 (75%) were receiving anticoagulation therapy with warfarin. Patients didn´t have severe alterations of calcium metabolism, all had product calcium-phosphorus < 55. All patients developed low blood pressure at the beginning of dialysis treatment (98.3 ± 22.7/60 ± 18,29 mmHg). 7 patients present proximal lesions in fatty regions like abdomen and thighs. Histopathologic examination reveals calcium deposits in arteriole-sized and small vessels with vascular thrombosis. Prognosis was poor, seven patients died secondary to a sepsis originated in infected cutaneous ulcers. Conclusions: calciphylaxis is a disease with poor prognosis and high mortality, without specific treatment actually. Female gender, obesity associated with diabetes mellitus and cardiometabolic syndrome, anticoagulant therapy with warfarin and low blood pressure associated with hemodialysis therapy, are risk factors to develop calciphylaxis, in absence of severe disorders of calcium metabolism. In these patients is important to avoid hypotension episodes during dialysis, dialysis hypotension appears to be an important risk factor who promotes ischemia of subcutaneous adipose tissue (AU)