Corticosteroids and mycophenolic acid analogues in immunoglobulin A nephropathy with progressive decline in kidney function.

Background: A randomized controlled trial demonstrated a beneficial effect of corticosteroids (CS) + cyclophosphamide followed by azathioprine in progressive immunoglobulin A nephropathy (IgAN). Although treatment with CS and mycophenolic acid analogues (MPAAs) remains controversial in IgAN, there is no information about their effects in progressive IgAN. Methods: Patients with progressive IgAN, defined by a decrease in estimated glomerular filtration rate (eGFR) of at least 10 mL/min/1.73 m2 in the 12 months prior to the start of treatment, proteinuria ≥0.75 g/24 h despite maximum tolerated doses of renin-angiotensin system blockers, and persistent haematuria who had received treatment with CS + MPAA were included in this retrospective study. The main outcome was the difference between the eGFR slope from the start of treatment with CS + MPAA to the last visit with this treatment with respect to the eGFR slope during the 12 months prior to the start of treatment. Results: A total of 25 patients were included in the study. The mean duration of CS + MPAA treatment was 24.7 ± 15.2 months. In the 12 months prior to treatment the median rate of kidney function decline was 23 mL/min/1.73 m2/year [interquartile range (IQR) -32 to -16]. After the onset of treatment, the median eGFR slope was 5 mL/min/1.73 m2/year (IQR 3-9; P = 0.001 with respect to the 12 months prior to treatment). Proteinuria decreased from 1.8 g/day (IQR 1.0-2.5) at baseline to 0.6 g/day (IQR 0.3-1.2) at the end of treatment (P = 0.01) and haematuria disappeared in 40% of patients. There were no serious adverse effects requiring treatment discontinuation. Conclusions: CS + MPAA is an effective treatment in IgAN patients with a sustained decline in kidney function accompanied by persistent proteinuria and haematuria despite optimized conservative treatment. Prospective studies are needed to confirm these results.

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.

Calcifilaxis: más allá de CKD-MBD / Calciphylaxis: Beyond CKD-MBD

Introducción: La arteriolopatía urémica calcificante (CUA), también llamada calcifilaxis, es un trastorno vascular poco frecuente pero potencialmente mortal que afecta casi exclusivamente a pacientes con insuficiencia renal crónica. El objetivo de este estudio fue analizar los diferentes factores de riesgo para el desarrollo de CUA y su posterior evolución según la terapia recibida. Material y métodos: Estudio retrospectivo que recoge aquellos pacientes con diagnóstico de CUA desde diciembre de 1999 hasta diciembre de 2015. Se analizaron diferentes factores de riesgo, evolución y diferentes opciones terapéuticas. Resultados: Se incluyeron 28 pacientes (53,6% mujeres) con una edad media de 67,2±11,8 años (38-88). En el momento del diagnóstico, el 53,6% estaba en hemodiálisis, un 25% eran pacientes con un trasplante renal y el 21,4% presentaba función renal normal. En los pacientes trasplantados, el consumo de esteroides (100%; p=0,001) fue el principal factor de riesgo. La resolución de lesiones cutáneas se produjo en el 60,7% (especialmente en los que recibieron tratamiento multitarget). La supervivencia de los pacientes a los 12meses fue de 29, 57 y 100% en los pacientes trasplantados, hemodiálisis y con función renal normal respectivamente (log-rank 6,88; p=0,032). La presencia de insuficiencia renal crónica (p=0,03) e hipoalbuminemia (p=0,02) fueron los principales factores de riesgo de mortalidad CUA. Conclusión: Aunque la incidencia de la CUA sigue siendo baja, su mortalidad es muy elevada, por lo que debe prestarse especial atención a la presentación de la CUA en los trasplantados renales y en las formas «no renales». Los anticoagulantes orales y los esteroides aparecen como principales factores de riesgo. La CUA es un reto: necesitamos un registro de nuestros pacientes y establecer una terapia estándar (AU)

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-renal» 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 (AU)

Nefropatía IgA: qué pacientes están en riesgo de progresar a enfermedad renal terminal y cómo deberían ser tratados? / IgA nephropathy: What patients are at risk of progression to end-stage renal disease and how should they be treated?

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