Resumen La endocarditis asociada a ANCA anti-PR3 e insuficiencia renal plantea un dilema tanto en su diagnóstico como tratamiento. Para abordar una revisión de dicho tema, se presenta el caso de un paciente con glomerulonefritis rápidamente progresiva secundaria a endocarditis subaguda por Enterococcus faecalis y positividad para ANCA anti-PR3. El diagnóstico diferencial principal se establecería entre una afectación renal de una vasculitis asociada a ANCA no diagnosticada previamente vs una glomerulonefritis postinfecciosa secundaria a la endocarditis. En muchos casos es necesario disponer de una biopsia renal que esclarezca el diagnóstico, ya que una inmunofluorescencia negativa orientará hacia una vasculitis, mientras que una positividad para inmunocomplejos iría a favor de una glomerulonefritis postestreptocócica. El tratamiento a seguir es otro reto que se plantea en la coexistencia de insuficiencia renal aguda, endocarditis y ANCA anti-PR3 positivo. La positividad de ANCAs induce a valorar iniciar tratamiento con inmunosupresores, no obstante, en el lecho de una endocarditis puede resultar desaconsejado e incluso poner en riesgo la vida del paciente someterlo a un estado de inmunosupresión. Es, por tanto, controvertido el uso de inmunosupresión en combinación con antibioterapia en el proceso agudo en contraposición al uso de antibioterapia exclusivamente. En el actual artículo se recogen los 19 casos publicados en la literatura de endocarditis asociados a ANCA anti-PR3, así como el tratamiento que se realizó en cada uno de los casos y la evolución en la función renal de cada paciente, concluyendo, en general, una mejor recuperación de la función renal en los pacientes tratados con antibioterapia en exclusiva que en aquellos tratados con la combinación antibiótico-corticoides. Sin embargo, dado el pequeño tamaño muestral, no se puede considerar significativa la diferencia entre ambos tratamientos. Absctrat Endocarditis associated with antiPR3 ANCA and acute kidney injure generates a challenge in its diagnosis and treatment. In order to make a review about that combination, we presented a patient with necrotizing glomerulonephritis produced by a Enterococcus faecalis´s subacute endocarditis and antiPR3 ANCA positive. Differential diagnosis is made between an acute kidney failure produced by ANCA´s vasculitis vs necrotizing glomerulonephritis by endocarditis. Frequently it is necessary to make a biopsy to get a diagnosis. Negative immunofluorescence will guide to vasculitis associated ANCA, while positive immune complexes will guide to poststreptococcal glomerulonephritis. Other challenge that generates the association of acute kidney disease, endocarditis and antiPR3 ANCA is the treatment. ANCA positive can prompt to start immunosuppressant treatments. However, in the context of endocarditis, it could be inadvisable and even dangerous to use it. For this reason, it is controversial the use of immunosuppressant in combination with antibiotics in the acute process, in contrast with the use of only antibiotics....

BACKGROUND AND AIMS Total kidney volume (TKV) in patients with autosomal dominant polycystic kidney disease (ADPKD) is a known progression factor in these patients. Low levels of klotho and high levels of FGF23 in the blood of patients with ADPKD have been correlated with higher TKV even in early stages of the disease, even with glomerular filtration rate (eGFR) >90 mL/min/1.73 m2. Therefore, alterations in the levels of parathyroid hormone (PTH), vitamin D, tubular reabsorption of phosphates or urinary calcium could be correlated with the TKV and serve as an early serum biomarker of progression of kidney disease. METHOD Observational, cross-sectional, single-center study in a population of 87 patients under follow-up at our center during 2019–2020 for ADPKD. The TKV, obtained by the ellipsoid formula measured by magnetic resonance imaging (MRI) and the values of PTH, vitamin D, tubular phosphate reabsorption and urinary calcium were retrospectively collected. Subsequently, a statistical analysis of correlation between the different variables was performed. RESULTS Spearman's Rho correlation analysis obtained a positive correlation of moderate–strong intensity between the TKR of the sample and PTH levels (r = 0.638, P <0 0.0001) and a negative correlation of moderate intensity with tubular phosphate reabsorption (r = −0.479, P < 0.005). No statistically significant correlation was obtained between the TKV and the levels of urinary calcium or serum vitamin D. TKV was higher among patients with an eGFR >90 mL/min/1.73 m2 (CKD-EPI) with an elevated PTH (> 65 pg/mL), with a median of 530 mL (interquartile range 403–1184 mL), compared with the TKV of those patients with an eGFR >90 mL/min/1.73 m2 with normal PTH, whose median was 405 mL (interquartile range 258–576 mL). Despite this trend, the Mann–Whitney U test did not show a statistically significant difference between medians in the subgroup of patients with eGFR >90 mL/min/1.73 m2. However, the comparison of medians in the subgroup of patients with eGFR <90 mL/min/1.73 m2 was statistically significant with a P < 0.019, with higher TKR in those patients with high PTH. CONCLUSION The increase in PTH in patients with ADPKD has a strong positive correlation with TKV. Even with normal eGFR, elevated PTH is associated with a higher TKV, which could be an early serum biomarker of kidney disease progression in this group of patients.

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BACKGROUND AND AIMS ANCA-associated vasculitis (AAV) is an autoimmune disease that causes end-stage renal disease in ∼20%–25% of patients who develop kidney failure with progression to end-stage kidney disease with requirement of renal replacement therapy. Previous studies have found a genetic association between some major histocompatibility complex (MHC) loci, like the locus DPB1*0401, and AAV. We conducted a retrospective observational unicentric study with the objective to identify other possible loci related to AAV. METHOD As MHC determination is not usually done in patients with AAV, we looked for patients with AAV included in the kidney transplant list with a complete classic HLA allele determination registered (A, B, C, DR, and DQ). Only 14 patients with diagnosis of AAV included in the kidney transplant list from August 1993 to December 2020 in our hospital centre fulfilled this inclusion criterion. To the statistical analysis, we compared the observed proportion to the theoretical one using a normal test, since the groups in our sample included at least five subjects. We also use a continuity correction factor to approach to a binomial test. RESULTS The highest allele frequency in our cohort were the HLA B*51and the haplotype HLA DRB1*04-DQB1*03:02. We identified a B*51 frequency of 42.8% (6/14 patients) in our AAV register, while its population frequency in Spain is 15%, difference = 27.86% [Confidence interval (CI) 1.93%–53.78%] P = .01. We detected a DRB1*04 frequency of 57.1% (8/14 patients) in our cohort, while the population frequency of DRB1*04 and its associated loci DQB1*03:02 is 22%, difference = 35.14% (CI 9.22%–61.07%) P < .05. CONCLUSION HLA B*51 and the haplotype DRB1*04-DQB1*03:02 are alleles more frequent in patients with AAV than general population. This could translate a genetic predisposition of people with these alleles to develop AAV.