IgA vasculitis with nephritis (Henoch-Schönlein purpura) after COVID-19: A case series and review of the literature.

COVID-19 most related glomerular disease to date seems to be collapsing glomerulopathy, mostly in young Afroamerican patients with APOL1 gene risk alleles. However, in our population, predominant in elderly Caucasian patients, most biopsied pathology since the beginning of the pandemic has been IgA nephritis or Schönlein-Henoch purpura. Since the description of the first case of this entity after SARS-CoV-2 infection by our research group, three more cases have arisen, which are described in the following article. In contrast to the rest of IgA vasculitis cases reported, our patients presented more renal function deterioration and all of them required immunosupresive therapy. Moreover, some showed incomplete recovery of renal function. This case series strengthens the hypothesis that SARS-CoV-2 infection may be another trigger of this pathology.

Vasculitis IgA con nefritis (púrpura de Schönlein-Henoch) tras COVID-19: una serie de casos y revisión de la literatura / IgA vasculitis with nephritis (Henoch-Schönlein purpura) after COVID-19: A case series and review of the literature

La patología glomerular más relacionada con enfermedad COVID-19 hasta la fecha parece ser la glomerulopatía colapsante, principalmente en pacientes de raza afroamericana y con alelos de riesgo para el gen APOL1. No obstante, en nuestra población, conformada por pacientes adultos mayores de raza caucásica, la patología más biopsiada desde el inicio de la pandemia ha sido la nefritis IgA o púrpura de Schönlein-Henoch.Desde la descripción del primer caso de esta entidad tras infección por SARS-CoV-2 por nuestro grupo de investigación hemos objetivado otros tres, los cuales se describen a continuación. En contraste con el resto de los casos publicados de vasculitis IgA, nuestros pacientes presentaban mayor deterioro de función renal y todos requirieron tratamiento inmunosupresor. Además, algunos presentaron recuperación incompleta de función renal. Esta serie de casos afianza la posibilidad de que la infección por SARS-CoV-2 sea un desencadenante más de esta patología. (AU)

COVID-19 most related glomerular disease to date seems to be collapsing glomerulopathy, mostly in young Afroamerican patients with APOL1 gene risk alleles. However, in our population, predominant in elderly Caucasian patients, most biopsied pathology since the beginning of the pandemic has been IgA nephritis or Schönlein-Henoch purpura.Since the description of the first case of this entity after SARS-CoV-2 infection by our research group, three more cases have arisen, which are described in the following article. In contrast to the rest of IgA vasculitis cases reported, our patients presented more renal function deterioration and all of them required immunosupresive therapy. Moreover, some showed incomplete recovery of renal function.This case series strengthens the hypothesis that SARS-CoV-2 infection may be another trigger of this pathology. (AU)

[IgA vasculitis with nephritis (Henoch-Schönlein purpura) after COVID-19: A case series and review of the literature]. / Vasculitis IgA con nefritis (púrpura de Schönlein-Henoch) tras COVID-19: una serie de casos y revisión de la literatura.

COVID-19 most related glomerular disease to date seems to be collapsing glomerulopathy, mostly in young Afroamerican patients with APOL1 gene risk alleles. However, in our population, predominant in elderly Caucasian patients, most biopsied pathology since the beginning of the pandemic has been IgA nephritis or Schönlein-Henoch purpura.Since the description of the first case of this entity after SARS-CoV-2 infection by our research group, three more cases have arisen, which are described in the following article. In contrast to the rest of IgA vasculitis cases reported, our patients presented more renal function deterioration and all of them required immunosupresive therapy. Moreover, some showed incomplete recovery of renal function.This case series strengthens the hypothesis that SARS-CoV-2 infection may be another trigger of this pathology.

Phenotypic characterization of X-linked hypophosphatemia in pediatric Spanish population.

BACKGROUND: X-linked hypophosphatemia (XLH) is a hereditary rare disease caused by loss-of-function mutations in PHEX gene leading tohypophosphatemia and high renal loss of phosphate. Rickets and growth retardation are the major manifestations of XLH in children, but there is a broad phenotypic variability. Few publications have reported large series of patients. Current data on the clinical spectrum of the disease, the correlation with the underlying gene mutations, and the long-term outcome of patients on conventional treatment are needed, particularly because of the recent availability of new specific medications to treat XLH. RESULTS: The RenalTube database was used to retrospectively analyze 48 Spanish patients (15 men) from 39 different families, ranging from 3 months to 8 years and 2 months of age at the time of diagnosis (median age of 2.0 years), and with XLH confirmed by genetic analysis. Bone deformities, radiological signs of active rickets and growth retardation were the most common findings at diagnosis. Mean (± SEM) height was - 1.89 ± 0.19 SDS and 55% (22/40) of patients had height SDS below-2. All cases had hypophosphatemia, serum phosphate being - 2.81 ± 0.11 SDS. Clinical manifestations and severity of the disease were similar in both genders. No genotype-phenotype correlation was found. Conventional treatment did not attenuate growth retardation after a median follow up of 7.42 years (IQR = 11.26; n = 26 patients) and failed to normalize serum concentrations of phosphate. Eleven patients had mild hyperparathyroidism and 8 patients nephrocalcinosis. CONCLUSIONS: This study shows that growth retardation and rickets were the most prevalent clinical manifestations at diagnosis in a large series of Spanish pediatric patients with XLH confirmed by mutations in the PHEX gene. Traditional treatment with phosphate and vitamin D supplements did not improve height or corrected hypophosphatemia and was associated with a risk of hyperparathyroidism and nephrocalcinosis. The severity of the disease was similar in males and females.

Duration of Treatment with Corticosteroids and Recovery of Kidney Function in Acute Interstitial Nephritis.

BACKGROUND AND OBJECTIVES: Drug-induced acute interstitial nephritis represents an emerging cause of acute kidney disease, especially among polymedicated elderly patients. Although corticosteroids are frequently used, controversy exists about the timing of initiation, efficacy, safety, and duration of treatment. DESIGN, SETTING, PARTICIPANTS, & MEASUREMENTS: We performed a retrospective study of 182 patients with biopsy-proven drug-induced acute interstitial nephritis from 13 Spanish centers. Exposure was defined as the length of corticosteroid treatment. The main outcome was the level of serum creatinine at month 6, with respect to baseline values. RESULTS: The most common offending agents were nonsteroidal anti-inflammatory drugs (27%). In 30% of patients, the offending drug could not be identified. The median time to suspected drug withdrawal was 11 days (interquartile range, 5-22). All patients presented with acute kidney disease and were treated with corticosteroids. The mean initial dose of prednisone was 0.8±0.2 mg/kg per day. High-dose corticosteroid treatment was maintained for 2 weeks (interquartile range, 1-4). After 6 months, the mean recovered GFR was 34±26 ml/min per 1.73 m2 and ten patients required maintenance dialysis. Use of high-dose corticosteroids for 3 weeks or treatment duration >8 weeks were not associated with better recovery of kidney function. In the multivariable analysis, delayed onset of steroid treatment (odds ratio, 1.02; 95% confidence interval, 1.0 to 1.04) and the presence of interstitial fibrosis of >50% on the kidney biopsy specimen (odds ratio, 8.7; 95% confidence interval, 2.7 to 27.4) were both associated with serum creatinine level at month 6 of >75%, with respect to baseline values. CONCLUSIONS: High-dose corticosteroid treatment for 3 weeks or prolonged treatment for >8 weeks were not associated with greater kidney function recovery in drug-induced acute interstitial nephritis. A delay in the initiation of corticosteroids resulted in worse recovery of kidney function.

Echocardiographic findings in haemodialysis patients according to their state of hydration / Hallazgos ecocardiográficos en pacientes en hemodiálisis según su estado de hidratación

Background: Chronic fluid overload is frequent in hemodialysis patients (P) and it associates with hypertension, left ventricular hypertrophy (LVH) and higher mortality. Moreover, echocardiographic data assessing fluid overload is limited. Our aim was to evaluate the relationship between fluid overload measured by bioimpedance spectroscopy (BIS) and different echocardiographic parameters. Methods: Cross-sectional observational study including 76 stable patients. Dry weight was clinically assessed. BIS and echocardiography were performed. Weekly time-averaged fluid overload (TAFO) and relative fluid overload (FO/ECW) were calculated using BIS measurements. Results: Based on TAFO three groups were defined: A- dehydrated, TAFO <-0.25 L 32 P (42%); B- normohydrated, TAFO between -0.25 and 1.5 l: 26 (34%); C- overhydrated, TAFO>1.5 l: 18 (24%). We found significant correlation between TAFO and left atrial volume index (LAVI) (r: 0.29; p=0.013) but not with FO/ECW (r 0.06; p=0.61). TAFO, but not FO/ECW kept a significant relationship with LAVI (p=0.03) using One-Way ANOVA test and linear regression methods. LVH was present in 73.7% (concentric 63.2%, eccentric in 10.5%). No differences between groups in the presence of LVH or left ventricular mass index were found. Conclusions: We found that left atrial volume index determined by echocardiographic Area-length method, but not left ventricle hypertrophy or dimensions of cavities, are related on hydration status based on bioimpedance measured time-averaged fluid overload (TAFO), and not with FO/ECW (AU)

Introducción: La sobrehidratación es frecuente en pacientes en hemodiálisis (P) y se asocia con hipertensión, hipertrofia ventricular izquierda (LVH) y mayor mortalidad. Los datos ecocardiográficos evaluando sobrecarga hídrica son escasos. Nuestro objetivo fue evaluar la relación entre sobrehidratación medida por Bioimpedancia multifrecuencia (BIS) y parámetros ecocardiográficos. Métodos: Estudio transversal observacional, con 76 P estables; El peso seco fue determinado clínicamente; se realizaron ecocardiograma, BIS y analítica sanguínea. Se calcularon la sobrehidratación promedio semanal (TAFO) y sobrehidratación relativa (FO/ECW). Resultados: 3 grupos: A- deshidratados, TAFO <-0.25 L: 32 P (42,1%); B- normohidratado, TAFO -0.25 - 1.5 L: 26 P (34,2%); C- sobrehidratados TAFO > 1.5 L: 18 P (23,7%). Encontramos correlación significativa entre TAFO e índice de volumen auricular izquierdo (LVAI) (r: 0.29; p=0.013) y no con FO/ECW (rho 0,06; p = 0,61). TAFO, pero no FO/ ECW, mantuvo una relación significativa con LVAI (p = 0,03) utilizando test de ANOVA y regresión lineal. LVH estuvo presente en 73,7% de P (concéntrica 63,2%, excéntrica 10,5%). No encontramos diferencias entre grupos en cuanto a la presencia de LVH, ni del índice de masa ventricular izquierda. Conclusiones: Nosotros observamos que el índice de volumen auricular izquierdo determinado por longitud de área medida por ecocardiograma y no la hipertrofia ventricular izquierda o dimensión de cavidades se relaciona con el estado de hidratación medido por sobrehidatación semanal y no con FO/ECW (AU)

Echocardiographic findings in haemodialysis patients according to their state of hydration.

BACKGROUND: Chronic fluid overload is frequent in hemodialysis patients (P) and it associates with hypertension, left ventricular hypertrophy (LVH) and higher mortality. Moreover, echocardiographic data assessing fluid overload is limited. Our aim was to evaluate the relationship between fluid overload measured by bioimpedance spectroscopy (BIS) and different echocardiographic parameters. METHODS: Cross-sectional observational study including 76 stable patients. Dry weight was clinically assessed. BIS and echocardiography were performed. Weekly time-averaged fluid overload (TAFO) and relative fluid overload (FO/ECW) were calculated using BIS measurements. RESULTS: Based on TAFO three groups were defined: A- dehydrated, TAFO <-0.25 L 32 P (42%); B- normohydrated, TAFO between -0.25 and 1.5 l: 26 (34%); C- overhydrated, TAFO>1.5 l: 18 (24%). We found significant correlation between TAFO and left atrial volume index (LAVI) (r: 0.29; p=0.013) but not with FO/ECW (r 0.06; p=0.61). TAFO, but not FO/ECW kept a significant relationship with LAVI (p=0.03) using One-Way ANOVA test and linear regression methods. LVH was present in 73.7% (concentric 63.2%, eccentric in 10.5%). No differences between groups in the presence of LVH or left ventricular mass index were found. CONCLUSIONS: We found that left atrial volume index determined by echocardiographic Area-length method, but not left ventricle hypertrophy or dimensions of cavities, are related on hydration status based on bioimpedance measured time-averaged fluid overload (TAFO), and not with FO/ECW.

Glomerulonephritis and cryoglobulinemia: first manifestation of visceral leishmaniasis.

Visceral leishmaniasis due to Leishmania Infantum is an endemic parasitic infection in the Mediterranean area. Since 2009, Europe's largest outbreak of Leishmaniasis has been reported in the region of Madrid (Spain). Renal involvement is an unusual complication. Different forms of renal disease have been described: interstitial, glomerular, and vascular damage. Direct invasion of renal parenchyma by the parasite has been described as a mechanism of kidney damage, especially in the immunocompromised. Immune complex deposition and T cells adhesion molecules activation have demonstrated that a pathogenic role in glomerulonephritis related to visceral leishmaniasis. The association between mixed cryoglobulinemia and visceral leishmaniasis has been previously reported in six patients. Renal involvement is only described in one of them. From July 2009 to October 2012, 4 patients with membranoproliferative glomerulonephritis and mixed cryoglobulinemia with negative serology for hepatitis B and C were diagnosed in our hospital. Serology of Leishmania in serum bank samples was performed; it was positive in 3 patients. Leishmania parasite was confirmed by other tests. We present 3 patients with mixed cryoglobulinemia and membranoproliferative glomerulonephritis as first clinical manifestation of visceral leishmaniasis.

Predictors of response and relapse in patients with idiopathic membranous nephropathy treated with tacrolimus.

BACKGROUND: Although tacrolimus is recommended by KDIGO Clinical Practice Guideline for Glomerulonephritis for the treatment of idiopathic membranous nephropathy (MN), little is known about factors that influence response and relapse of the disease after tacrolimus therapy. METHODS: Multicentre study that collected 122 MN patients with nephrotic syndrome and stable renal function treated with tacrolimus. Duration of treatment was 17.6 ± 7.2 months, including a full-dose and a tapering period. RESULTS: The percentage of remission was 60, 78 and 84% after 6, 12 and 18 months of treatment, respectively. The amount of proteinuria at baseline significantly predicted remission, the lower the baseline proteinuria the higher the probability of remission. Only 10 patients (8%) received concomitantly corticosteroids, and their rate of remission was similar (80% at 18 months). Among responders, 42% achieved complete remission (CR) and 58% partial remission (PR). Almost half (44%) of the responder patients relapsed. The amount of proteinuria at the onset of tacrolimus tapering was significantly higher in relapsing patients. By multivariable analysis, the presence of a PR versus CR at the onset of tacrolimus tapering and a shorter duration of the tapering period significantly predicted relapses. Tolerance was good and the number of adverse events low. CONCLUSIONS: Tacrolimus monotherapy is an effective and safe option for the treatment of MN with stable renal function. Relapses are frequent in patients with PR and can be partially prevented by a longer tapering period.