Prophylactic Anticoagulation Reduces the Risk of Kidney Graft Venous Thrombosis in Recipients From Uncontrolled Donation After Circulatory Death Donors With High Renal Resistive Index.

Background: Uncontrolled donation after circulatory death (uDCD) increases organ availability for kidney transplantation (KT) at the expense of a higher risk of primary graft nonfunction (PNF). At least half of the cases of PNF are secondary to graft venous thrombosis. The potential benefit from prophylactic anticoagulation in this scenario remains unclear. Methods: In this single-center retrospective study we compared 2 consecutive cohorts of KT from uDCD with increased (≥0.8) renal resistive index (RRI) in the Doppler ultrasound examination performed within the first 24-72 h after transplantation: 36 patients did not receive anticoagulation ("nonanticoagulation group") and 71 patients underwent prophylactic anticoagulation until normalization of RRI in follow-up Doppler examinations ("anticoagulation group"). Results: Anticoagulation was initiated at a median of 2 d (interquartile range, 2-3) after transplantation and maintained for a median of 12 d (interquartile range, 7-18). In 4 patients (5.6%), anticoagulation had to be prematurely stopped because of the development of a hemorrhagic complication. In comparison with the nonanticoagulation group, recipients in the anticoagulation group had a lower 2-wk cumulative incidence of graft venous thrombosis (19.4% versus 0.0%; P < 0.001) and PNF (19.4% versus 2.8%; P = 0.006). The competing risk analysis with nonthrombotic causes of PNF as the competitive event confirmed the higher risk of graft thrombosis in the nonanticoagulation group (P = 0.0001). The anticoagulation group had a higher incidence of macroscopic hematuria (21.1% versus 5.6%; P = 0.049) and blood transfusion requirements (39.4% versus 19.4%; P = 0.050) compared with the nonanticoagulation group. No graft losses or deaths were attributable to complications potentially associated with anticoagulation. Conclusions: Early initiation of prophylactic anticoagulation in selected KT recipients from uDCD with an early Doppler ultrasound RRI of ≥0.8 within the first 24-72 h may reduce the incidence of graft venous thrombosis as a cause of PNF.

Vitamina D, magnesio y parámetros seleccionados de laboratorio en hombres uruguayos / Vitamin D, magnesium, and selected laboratory parameters in Uruguayan men / Vitamina D, magnésio e parâmetros selecionados de laboratório em homens uruguaios

Resumen: Introducción: los niveles de vitamina D (VD) plasmática se han investigado sobre todo en mujeres, no obstante también son relevantes en hombres, y no sólo en relación al estado de salud ósea. Un estudio previo realizado hace dos décadas en una pequeña muestra de hombres y mujeres uruguayos reveló serias deficiencias de VD. Por otra parte, el magnesio posee importantes efectos no solo relacionados a los niveles de VD, también afecta el hueso directa e indirectamente. Objetivos: analizar posibles interrelaciones entre valores de VD plasmática, testosterona plasmática, densitometría ósea, y otros parámetros en una población de jóvenes varones uruguayos. Métodos: durante julio-agosto de 2015, cien hombres uruguayos afiliados al sistema prepago de salud fueron invitados a participar en nuestra Unidad de Metabolismo Fosfocálcico, para ser estudiados con técnicas de antropometría, laboratorio y densitométricas. Los datos colectados fueron analizados estadísticamente. Resultados: los hombres estudiados mostraron bajos niveles de VD plasmática (96% insuficientes-deficientes), que no mostraron correlaciones significativas con ninguno de los parámetros estudiados. El magnesio plasmático se correlacionó significativa e inversamente con los z-scores densitométricos, lo que fue más intenso en hombres con sobrepeso y con VD ≥15 ng/ml. Conclusiones: a pesar de lo pequeño de la muestra, los resultados confirman la presencia de bajos niveles de VD en hombres aparentemente saludables. Los hallazgos dan razones para pensar en posibles recomendaciones para mejorar el espectro preventivo enfocado en osteoporosis y cáncer colorrectal.

Summary: Background: serum vitamin D (VD) levels have been mainly studied in women, but they are relevant also among men, not only regarding bone health status. A previous small-size study performed two decades ago in Uruguayan women and men revealed serious deficiencies of VD. On the other hand, magnesium has important effects not only related to vitamin D levels but directly and indirectly affect bone. Purpose: to analyze possible interrelationships among values of serum VD, magnesium, testosterone, bone densitometry and other parameters in young Uruguayan male population. Methods: during July-August 2015, one hundred Uruguayan men affiliated to the prepaid healthcare system, invited to participate by our Unit of Bone Metabolism, were studied with anthropometry, laboratory and, densitometry techniques. Collected data were statistically analyzed. Results: the studied men showed low serum VD levels (96% insufficient-deficient), which did not show significant correlations with any of the studied parameters. Serum magnesium was significantly and inversely correlated with densitometric z-scores, even stronger among men with overweight and with VD≥15 ng/ml. Conclusions: despite the sample size, results confirm the presence of low serum VD levels in apparently healthy men. Findings give reasons to think about possible recommendations to improve the preventive scope regarding osteoporosis and colorectal cancer.

Resumo: Introdução: os níveis plasmáticos de vitamina D (DV) têm sido investigados principalmente em mulheres, porém também são relevantes em homens, e não apenas em relação à saúde óssea. Um estudo anterior realizado há duas décadas em uma pequena amostra de homens e mulheres uruguaios revelou graves deficiências de VD. Por outro lado, o magnésio tem efeitos importantes não apenas relacionados aos níveis de VD, mas também afeta o osso direta e indiretamente. Objetivos: analisar as possíveis relações entre os valores plasmáticos de VD, testosterona plasmática, densitometria óssea e outros parâmetros em uma população de jovens uruguaios. Métodos: durante o período de julho a agosto de 2015, cem uruguaios filiados ao sistema de saúde pré-pago foram convidados a participar de nossa Unidade de Metabolismo Fosfocálcico, para serem estudados com técnicas antropométricas, laboratoriais e densitométricas. Os dados coletados foram analisados estatisticamente. Resultados: os homens estudados apresentaram baixos níveis de VD plasmático (96% insuficiente-deficiente), que não apresentou correlações significativas com nenhum dos parâmetros estudados. O magnésio plasmático foi significativa e inversamente correlacionado com os escores z densitométricos, que foi mais intenso em homens com sobrepeso e VR ≥15 ng / ml. Conclusões: porém, na pequena amostra, os resultados confirmam a presença de baixos níveis de VD em homens aparentemente saudáveis. Estes achados propõem pensar sobre possíveis recomendações para melhorar o espectro preventivo com foco na osteoporose e no câncer colorretal.

Kidney transplantation in the extremely elderly from extremely aged deceased donors: a kidney for each age.

BACKGROUND: Advances in life expectancy have led to an increase in the number of elderly people with end-stage renal disease (ESRD). Scarce information is available on the outcomes of kidney transplantation (KT) in extremely elderly patients based on an allocation policy prioritizing donor-recipient age matching. METHODS: We included recipients ≥75 years that underwent KT from similarly aged deceased donors at our institution between 2002 and 2015. Determinants of death-censored graft and patient survival were assessed by Cox regression. RESULTS: We included 138 recipients with a median follow-up of 38.8 months. Median (interquartile range) age of recipients and donors was 77.5 (76.3-79.7) and 77.0 years (74.7-79.0), with 22.5% of donors ≥80 years. Primary graft non-function occurred in 8.0% (11/138) of patients. Cumulative incidence rates for post-transplant infection and biopsy-proven acute rejection (BPAR) were 70.3% (97/138) and 15.2% (21/138), respectively. One- and 5-year patient survival were 82.1 and 60.1%, respectively, whereas the corresponding rates for death-censored graft survival were 95.6 and 93.1%. Infection was the leading cause of death (46.0% of fatal cases). The occurrence of BPAR was associated with lower 1-year patient survival [hazard ratio (HR) = 4.21, 95% confidence interval (CI) 1.64-10.82; P = 0.003]. Diabetic nephropathy was the only factor predicting 5-year death-censored graft survival (HR = 4.82, 95% CI 1.08-21.56; P = 0.040). CONCLUSIONS: ESRD patients ≥75 years can access KT and remain dialysis free for their remaining lifespan by using grafts from extremely aged deceased donors, yielding encouraging results in terms of recipient and graft survival.

Substitution of Oral for Intravenous Cyclophosphamide in Membranous Nephropathy.

Background: Optimal immunosuppressive treatment for membranous nephropathy is still a matter of controversy. Current recommendations include oral cyclophosphamide combined with steroids (modified Ponticelli regimen) as first-line treatment in patients who are high risk. However, concerns about the cumulative toxicity of oral cyclophosphamide persist. In the last 30 years, a protocol based on low-dose intravenous cyclophosphamide plus steroids has been used to treat membranous nephropathy in Uruguay. We aimed to assess the efficacy of this regimen to induce clinical remission in patients with membranous nephropathy. Methods: In this retrospective, observational cohort study, we analyzed the outcome of 55 patients with membranous nephropathy treated between 1990 and 2017 with a 6-month course of alternating steroids (months 1, 3, and 5) plus intravenous cyclophosphamide (single dose of 15 mg/kg on the first day of months 2, 4, and 6). Results: At 24 months, 39 (71%) patients achieved clinical response with complete remission observed in 23 patients (42%) and partial remission in 16 (29%). Median time to achieve partial and complete remission was 5.9 and 11.5 months, respectively. Absence of response was observed in 16 patients (29%), five of whom started chronic RRT after a median follow-up of 3.5 years. Clinical relapse occurred in nine of 33 (27%) patients at a median of 34 months after treatment discontinuation. Conclusions: Replacement of oral cyclophosphamide with a single intravenous pulse on months 2, 4, and 6 of the modified Ponticelli regimen can be an effective and safe alternative for treatment of membranous nephropathy. Podcast: This article contains a podcast at https://www.asn-online.org/media/podcast/K360/2020_09_24_KID0002802020.mp3.

IgA Nephropathy in Elderly Patients.

BACKGROUND AND OBJECTIVES: Some studies suggest that the incidence of IgA nephropathy is increasing in older adults, but there is a lack of information about the epidemiology and behavior of the disease in that age group. DESIGN, SETTING, PARTICIPANTS, & MEASUREMENTS: In this retrospective multicentric study, we analyzed the incidence, forms of presentation, clinical and histologic characteristics, treatments received, and outcomes in a cohort of 151 patients ≥65 years old with biopsy-proven IgA nephropathy diagnosed between 1990 and 2015. The main outcome was a composite end point of kidney replacement therapy or death before kidney replacement therapy. RESULTS: We found a significant increase in the diagnosis of IgA nephropathy over time from six patients in 1990-1995 to 62 in 2011-2015 (P value for trend =0.03). After asymptomatic urinary abnormalities (84 patients; 55%), AKI was the most common form of presentation (61 patients; 40%). Within the latter, 53 (86%) patients presented with hematuria-related AKI (gross hematuria and tubular necrosis associated with erythrocyte casts as the most important lesions in kidney biopsy), and eight patients presented with crescentic IgA nephropathy. Six (4%) patients presented with nephrotic syndrome. Among hematuria-related AKI, 18 (34%) patients were receiving oral anticoagulants, and this proportion rose to 42% among the 34 patients older than 72 years old who presented with hematuria-related AKI. For the whole cohort, survival rates without the composite end point were 74%, 48%, and 26% at 1, 2, and 5 years, respectively. Age, serum creatinine at presentation, and the degree of interstitial fibrosis in kidney biopsy were risk factors significantly associated with the outcome, whereas treatment with renin-angiotensin-aldosterone blockers was associated with a lower risk. Immunosuppressive treatments were not significantly associated with the outcome. CONCLUSIONS: The diagnosis of IgA nephropathy among older adults in Spain has progressively increased in recent years, and anticoagulant therapy may be partially responsible for this trend. Prognosis was poor. PODCAST: This article contains a podcast at https://www.asn-online.org/media/podcast/CJASN/2019_07_16_CJASNPodcast_19_08_.mp3.

Kidney transplant from uncontrolled donation after circulatory death donors maintained by nECMO has long-term outcomes comparable to standard criteria donation after brain death.

Uncontrolled donation after circulatory death (uDCD) increases organ availability for kidney transplant (KT) with short-term outcomes similar to those obtained from donation after brain death (DBD) donors. However, heterogeneous results in the long term have been reported. We compared 10-year outcomes between 237 KT recipients from uDCD donors maintained by normothermic extracorporeal membrane oxygenation (nECMO) and 237 patients undergoing KT from standard criteria DBD donors during the same period at our institution. We further analyzed risk factors for death-censored graft survival in the uDCD group. Delayed graft function (DGF) was more common in the uDCD group (73.4% vs 46.4%; P < .01), although glomerular filtration rates at the end of follow-up were similar in the 2 groups. uDCD and DBD groups had similar rates for 10-year death-censored graft (82.1% vs 80.4%; P = .623) and recipient survival (86.2% vs 87.6%; P = .454). Donor age >50 years was associated with graft loss in the uDCD group (hazard ratio: 1.91; P = .058), whereas the occurrence of DGF showed no significant effect. uDCD KT under nECMO support resulted in similar graft function and long-term outcomes compared with KT from standard criteria DBD donors. Increased donor age could negatively affect graft survival after uDCD donation.

Temporal trends in biopsy proven glomerular disease in Uruguay, 1990-2014.

Our aim is to describe variations in the incidence rates of glomerular disease diagnosed by renal biopsies performed in Uruguay over the last 25 years in relation to sex, age, clinical presentation and histological diagnosis. We analyzed all renal biopsies performed in Uruguay during the 25 years period and estimated incidence rates per million people per year (pmp/yr) for the population older than 14 years. Mann Kendall's trend analysis was used to assess incidence trends. In order to identify changes in trends, we compared annual incidence rates with the Joinpoint method. From 1990 to 2014, 3390 biopsies of native kidneys corresponding to glomerular disease were performed in patients older than 14 years. The average biopsy rate was 58 per pmp/yr. The glomerular disease incidence rate increased progressively over the period (p<0.05). Trends analysis over five-year periods demonstrated a progressive increase of IgA nephropathy (3.08 pmp/yr 1990-1994 to 12.53 pmp/yr 2010-2014 p<0.05), membranous nephropathy (2.38 pmp/yr 1990-1994 to 8.04 pmp/yr 2010-2014 p< 0.05) and lupus nephritis (4,23 pmp/yr 1990-1994 to 7,81 pmp/yr 2010-2014 p<0.05). There was a change in the trend of focal segmental glomerular sclerosis (FSGS) which increased until 1996 and decreased afterwards. The incidence rates of glomerular disease have doubled globally in the last quarter of a century in Uruguay, mainly related to the increase of IgA nephropathy, membranous nephropathy and lupus nephritis. There was a change in the slope of the incidence rate of FSGS.

Nefropatía IgA en Uruguay: presentación clínica y evolución / IgA Nephropathy in Uruguay: clinical presentation and evolution

La nefropatía IgA es frecuente en Uruguay y su tratamiento conflictivo. Objetivo: analizar la incidencia, presentación clínica, histología, tratamiento y evolución de la nefropatía IgA en Uruguay. Material y método: se realizó un análisis retrospectivo de datos del Registro Uruguayo de Glomerulopatías (RUG) e historias clínicas con estricta confidencialidad; se registró edad, sexo, fecha de biopsia renal, presentación clínica, presión arterial (PA), proteinuria, hematuria y creatininemia (al inicio y en última evolución registrada), histología, tratamientos y evolución. Análisis estadístico uni y multivariado. Aprobado por el Comité de Ética del Hospital de Clínicas. Resultados: se estudiaron 341 pacientes con nefropatía IgA, confirmada por histología, en el período comprendido entre el 1º de enero de 1985 y el 31 de diciembre de 2009; 65% hombres, edad media 31 ± 13 años y mediana de seguimiento de 52 meses (1-271); 14% ingresaron a diálisis y 1,2% fallecieron. La presentación clínica más frecuente fue con alteraciones urinarias asintomáticas (AUA) (42%), (mayor en el período 2000-2009, chi2 p < 0,05). Al inicio, la proteinuria era 1,7 ± 1,9 g/l; creatininemia 1,6 ± 1,8 mg/dl, y 32% tenían PA ≥ 140/90 mmHg. Se observaron semilunas en 48%. Recibieron inhibidores de la enzima convertidora de angiotensina/antagonistas de los receptores de la angiotensina II (IECA/ARAII) 56% e inmunosupresores, 52%. La PA disminuyó significativamente en la evolución. En el análisis univariado, la creatininemia inicial fue ≥ 2,5 mg/dl, proteinuria, semilunas y proliferación endocapilar se asociaron a ingreso a diálisis o fallecimiento, pero en el análisis multivariado (regresión de Cox) solo fue significativo el nivel de creatininemia. Conclusiones: la presentación clínica de nefropatía IgA ha cambiado en la última década, siendo más frecuente las AUA. La creatininemia ≥ 2,5 mg/dl se asocia a peor supervivencia renal, probable “punto de no retorno”...

IgA nephropathy is a frequent condition in Uruguay and treating it is controversial.Objective: to analyse incidence, clinical presentation, histology, treatment and evolution of nephropathy in Uruguay.Method: we conducted a retrospective study of data kept at the Uruguayan Registry of Glomerulopathies and medical records under strict confidentiality. Age, sex, date of the kidney biopsy, clinical presentation, blood pressure, proteinuria, hematuria and creatininemia (initial and in the last evolution recorded), histology, treatment and evolution were registered. Single-variate and multivariate statistic analysis were applied. The study was approved by the Ethics Committee of the Clínicas University Hospital.Results: Three hundred and forty one patients with IgA nephropathy – confirmed with histopathology analysis - were studied. Sixty five per cent were men, average age was 31 ± 13 year old and follow-up median 52 months (1-271); 14% started with dialysis and 1.2% died. The most common clinical presentation was asymptomatic urinary alterations (42%), (and this was more evident in the 2000-2009 period, chi2 p < 0.05). At the beginning, proteinuria was 1.7 ± 1.9 g/l; creatininemia 1.6 ± 1.8 mg/dl, and blood pressure was PA ≥ 140/90 mmHg in 32% of patients. Crescent formations were observed in 48%. Fifty six per cent of them received angiotensin-converting enzyme (ACE) / angiotensin receptor blockers and 52% of patients received immunosuppressants. Blood pressure significantly dropped in the evolution. In the single-variate analysis, initial creatininemia was ≥ 2.5 mg/dl, proteinuria, crescent formations and endocapillary proliferation were associated to starting dialysis or death, although in the multivariate analysis (Cox regression) only the level of creatininemia was significant.Conclusions: the clinical presentation of IgA nephropathy has changed in the past decade, being the asymtomatic urinary alterations the most frequent...

A nefropatia IgA é frequente no Uruguai e seu tratamento conflitivo.Objetivo: analisar a incidência, apresentação clínica, histologia, tratamento e evolução da nefropatia IgA no Uruguai.Material e método: uma análise retrospectiva dos dados do Registro Uruguaio de Glomerulopatias (RUG) e prontuários médicos com absoluta confidencialidade foi realizada; foram registrados idade, sexo, data da biopsia renal, apresentação clínica, pressão arterial (PA), proteinúria, hematúria e creatininemia (no inicio e na última evolução registrada), histologia, tratamentos e evolução. Análise estatística uni e multivariada. Aprovado pelo Comitê de Ética do Hospital das Clínicas.Resultados: foram estudados 341 pacientes com nefropatia IgA, confirmada por histologia, no período 1º de janeiro de 1985 - 31 de dezembro de 2009; 65% eram homens, idade média 31 ± 13 anos e mediana de seguimento de 52 meses (1-271); 14% ingressaram a diálise e 1,2% faleceu. A apresentação clínica mais frequente foi presença de alterações urinárias assintomáticas (AUA) (42%), (maior no período 2000-2009, chi2 p < 0,05). No inicio, a proteinuria era 1,7 ± 1,9 g/l; creatininemia 1,6 ± 1,8 mg/dl, e 32% apresentaram PA ≥ 140/90 mmHg. Foram observadas crescentes celulares em 48%. Receberam inibidores da enzima conversora da angiotensina/antagonistas dos receptores da angiotensina (IECA/ ARA) 56% e imunossupressores, 52%. A PA diminuiu significativamente na evolução. Na análise univariada, a creatininemia inicial foi ≥2,5 mg/dl, proteinúria, crescentes celulares e proliferação endocapilar estavam associados ao ingresso à diálise e ao falecimento, porém na análise multivariada (regressão de Cox) somente o nível de creatininemia foi significativo.Conclusões: a apresentação clínica da nefropatía IgA mostrou mudanças na última década, sendo mais frequente as AUA. A creatininemia ≥ 2,5 mg/dl está associada a pior supervivência renal, provável “ponto de não retorno”...

Hipertensión arterial maligna: una forma de presentación de la nefropatía IgA con mal pronóstico / Malignant hypertension: a type of IgA nephropathy manifestation with poor prognosis

Introducción: La hipertensión arterial maligna (HTAM) es una manifestación clínica poco frecuente de la nefropatía IgA (NIgA). Su prevalencia, patogenia y evolución son escasamente conocidas. Material y métodos: Estudio retrospectivo que muestra las características al diagnóstico y la evolución de 13 enfermos con NIgA demostrada por biopsia e HTAM (NIgA-HTAM) diagnosticados en nuestro centro. Resultados: La prevalencia de HTAM en nuestros pacientes con NIgA fue del 7 % (13/186). La edad media de los enfermos fue de 37 ± 12 años. El 84 % eran varones. La presión arterial media al debut del cuadro fue de 219 ± 32/132 ± 18 mmHg. Todos presentaban insuficiencia renal, con una creatinina sérica (Crs) media de 4,73 ± 3,12 mg/dl. Ningún enfermo presentaba datos clínicos de anemia hemolítica microangiopática. El daño histológico fue moderado, existiendo datos de microangiopatía trombótica en cuatro pacientes. Todos los enfermos se trataron con bloqueantes del sistema renina-angiotensina y dos recibieron esteroides. Pese a ello, la función renal se deterioró progresivamente en todos los casos. Al final del seguimiento un enfermo había muerto, diez estaban en diálisis y los dos restantes presentaban una insuficiencia renal crónica estadio 3b. La probabilidad de supervivencia renal fue del 69 % y del 35 % a los 3 y 6 años de seguimiento. Seis enfermos recibieron un trasplante renal: la NIgA recidivó en cuatro de ellos y uno presentó un nuevo episodio de HTAM asociado a síndrome HELLP. Conclusiones: La HTAM es una forma de presentación de la NIgA con un pronóstico renal muy desfavorable y para la que no disponemos de tratamientos eficaces en la actualidad (AU)

Introduction: Malignant hypertension (MHT) is an uncommon clinical manifestation of IgA Nephropathy (IgAN). Its prevalence, pathogenesis and evolution are not well known. Material and methods: We performed a descriptive and retrospective study to report the clinical characteristics and evolution of thirteen patients diagnosed as having IgA nephropathy by renal biopsy in our hospital who developed MHT (IgAN-MHT). Results: The prevalence of MHT in our IgAN patients was 7% (13/186). The mean age was 37±12 years and 84% were males. Mean systolic/diastolic blood pressure at presentation were 219±32/132±18mmHg, respectively. Renal function impairment was detected at admission in all the patients, with a mean serum creatinine of 4.73±3.12mg/dL. No patient showed analytical data that suggested thrombotic microangiopathy. Renal biopsies showed mild chronicity lesions and only four patients presented features of thrombotic microangiopathy. All patients were treated with renin-angiotensin-aldosterone blockers and two received steroids. They all showed a progressive loss of renal function. At the end of follow up one patient had died, ten were on chronic dialysis and two presented chronic kidney disease stage 3b. Renal survival was 69% and 35% at 3 and 6 years, respectively. Six patients received a kidney transplant: IgAN relapsed in four patients. One of them presented a new episode of MHT associated with a HELLP syndrome. Conclusions: Malignant hypertension is a form of IgAN clinical presentation having a remarkably worse renal outcome and without specific effective treatment

Malignant hypertension: a type of IgA nephropathy manifestation with poor prognosis.

INTRODUCTION:  Malignant hypertension (MHT) is an uncommon clinical manifestation of IgA Nephropathy (IgAN). Its prevalence, pathogenesis and evolution are not well known.  MATERIAL AND METHODS:  We performed a descriptive and retrospective study to report the clinical characteristics and evolution of thirteen patients diagnosed as having IgA nephropathy by renal biopsy in our hospital who developed MHT (IgAN-MHT).  RESULTS:  The prevalence of MHT in our IgAN patients was 7% (13/186). The mean age was 37±12 years and 84% were males. Mean systolic/diastolic blood pressure at presentation were 219±32/132±18mmHg, respectively. Renal function impairment was detected at admission in all the patients, with a mean serum creatinine of 4.73±3.12mg/dL. No patient showed analytical data that suggested thrombotic microangiopathy. Renal biopsies showed mild chronicity lesions and only four patients presented features of thrombotic microangiopathy. All patients were treated with renin-angiotensin-aldosterone blockers and two received steroids. They all showed a progressive loss of renal function. At the end of follow up one patient had died, ten were on chronic dialysis and two presented chronic kidney disease stage 3b. Renal survival was 69% and 35% at 3 and 6 years, respectively. Six patients received a kidney transplant: IgAN relapsed in four patients. One of them presented a new episode of MHT associated with a HELLP syndrome.  CONCLUSIONS:  Malignant hypertension is a form of IgAN clinical presentation having a remarkably worse renal outcome and without specific effective treatment.