Membranoproliferative glomerulonephritis: current histopathological classification, clinical profile, and kidney outcomes / Glomerulonefrite membranoproliferativa: classificação histopatológica atual, perfil clínico e desfechos renais

Abstract Introduction: Membranoproliferative glomerulonephritis (MPGN) is a rare glomerular disease with a variable prognosis. A new classification based on the presence or absence of immunoglobulins and complement deposits in immunofluorescence microscopy (IF) of kidney biopsy has recently been proposed. The objectives of the study were to determine and compare the clinical, laboratory, and histopathological characteristics of those with primary or secondary MPGN, reclassify the primary ones based on IF findings, and evaluate kidney outcomes. Methods: This was an observational retrospective cohort study carried out in a single center (UNIFESP), based on the data collected from medical records of patients followed from 1996 to 2019. Results: Of 53 cases of MPGN, 36 (67.9%) were classified as primary and 17 (32.1%) as secondary MPGN. Most patients were hypertensive (84.9%) and had edema (88.7%) and anemia (84.9%); 33 (91.7%) patients classified as primary MPGN were reclassified as immune-complex-mediated and 3 (8.3%) as complement-mediated. The secondary MPGN group had hematuria more frequently (p <0.001) and a higher prevalence of deposits of IgG (p = 0.02) and C1q (p = 0.003). Regarding the outcome, 39% of the patients achieved partial or complete remission. Lower initial serum albumin and higher initial 24-hour proteinuria were factors associated with worst renal prognosis. Conclusions: According to the new histological classification, the vast majority of MPGN cases were classified as being mediated by immune complexes. There were few differences between primary and secondary MPGN in relation to their clinical and laboratory characteristics.

Resumo Introdução: Glomerulonefrite membranoproliferativa (GNMP) é uma doença glomerular rara com prognóstico variável. Recentemente, foi proposta uma nova classificação baseada na presença ou ausência de imunoglobulinas e depósitos de complemento na microscopia de imunofluorescência (IF) da biópsia renal. Os objetivos do estudo foram determinar e comparar as características clínicas, laboratoriais e histopatológicas daqueles com GNMP primária ou secundária, reclassificar as primárias com base em achados da IF e avaliar os desfechos renais. Métodos: Este foi um estudo de coorte observacional retrospectivo realizado em centro único (UNIFESP), com base nos dados coletados de prontuários de pacientes acompanhados de 1996 a 2019. Resultados: Dos 53 casos de GNMP, 36 (67,9%) foram classificados como GNMP primária e 17 (32,1%) como GNMP secundária. A maioria dos pacientes era hipertensa (84,9%) e apresentava edema (88,7%) e anemia (84,9%); 33 (91,7%) pacientes classificados como GNMP primária foram reclassificados como mediados por imunocomplexo e 3 (8,3%) como mediados por complemento. O grupo de GNMP secundária apresentou mais frequentemente hematúria (p <0,001) e maior prevalência de depósitos de IgG (p = 0,02) e C1q (p = 0,003). Com relação ao desfecho, 39% dos pacientes alcançaram remissão parcial ou completa. Albumina sérica inicial mais baixa e proteinúria de 24 horas inicial mais elevada foram fatores associados a pior prognóstico renal. Conclusões: De acordo com a nova classificação histológica, a grande maioria dos casos de GNMP foram classificados como sendo mediados por imunocomplexos. Houve poucas diferenças entre GNMP primária e secundária em relação às suas características clínicas e laboratoriais.

Tuberculosis presenting as proliferative glomerulonephritis with monoclonal immunoglobulin deposits / Tuberculosis que se presenta como glomerulonefritis proliferativa con depósitos de inmunoglobulina monoclonal

Abstract Proliferative glomerulonephritis with monoclonal immunoglobulin deposits is defined as membranoproliferative glomerulonephritis like injury with monotypic Ig deposits restricted to a single light chain isotype.Here we present a patient who presented with hypocomplementemia and nephrotic syndrome, who was initially diagnosed with proliferative glomerulonephritis with monoclonal immunoglobulin deposits. He developed disseminated tuberculosis after a brief course of immunosuppression. Successful treatment of tuberculosis resulted in the complete remission of glomerular disease and the disappearance of monoclonal protein. Hence, we believe he had Tuberculosis-related proliferative glomerulonephritis with monoclonal immunoglobulin deposits. Treatment strategies have not been structured due to the rarity of the condition and lack of randomized trials. However, expert opinion suggests clone-based therapy. proliferative glomerulonephritis with monoclonal immunoglobulin deposits with a benign course without clone-based therapy has been reported. Patients seldom respond to classic immunosuppressants. Even some cases experience slowly progressive disease under angiotensin converting enzyme inhibition alone. There are also cases secondary to viral infections. Our case and the particular "benign" cases lead us to an intriguing proposition that proliferative glomerulonephritis with monoclonal immunoglobulin deposits might not be a single disease. A subset of patients may be experiencing infection-related or post-infectious glomerulonephritis presenting as proliferative glomerulonephritis with monoclonal immunoglobulin deposits.

Resumen La lesión similar a la glomerulonefritis membranoproliferativa con depósitos de Ig monotípicos restringidos a un isotipo de cadena ligera única se conoce actualmente como glomerulonefritis proliferativa con depósitos de inmunoglobulina monoclonal. A continuación presentamos a un paciente que presentó hipocomplementemia y síndrome nefrótico, al que inicialmente se le diagnosticó glomerulonefritis proliferativa con depósitos de inmunoglobulina monoclonal. Desarrolló tuberculosis diseminada después de un breve curso de inmunosupresión. El tratamiento exitoso de la tuberculosis dio como resultado la remisión completa de la enfermedad glomerular y la desaparición de la proteína monoclonal. Por lo tanto, creemos que tenía glomerulonefritis proliferativa con depósitos de inmunoglobulina monoclonal relacionada con tuberculosis diseminada. Las estrategias de tratamiento no se han estructurado debido a la rareza de la afección y la falta de ensayos aleatorios. Sin embargo, la opinión de los expertos sugiere una terapia basada en clones. Se ha informado de glomerulonefritis proliferativa con depósitos de inmunoglobulina monoclonal con un curso benigno sin terapia basada en clones. Los pacientes rara vez responden a los inmunosupresores clásicos. Incluso algunos casos experimentan una enfermedad de progresión lenta solo con la inhibición de la enzima convertidora de angiotensina. También hay casos secundarios a infecciones virales. Nuestro caso y los casos "benignos" particulares nos llevan a la propuesta intrigante de que la glomerulonefritis proliferativa con depósitos de inmunoglobulina monoclonal podría no ser una sola enfermedad. Un subgrupo de pacientes puede estar experimentando glomerulonefritis postinfecciosa o relacionada con una infección que se presenta como glomerulonefritis proliferativa con depósitos de inmunoglobulina monoclonal.

Abordaje clínico y diagnóstico de la glomerulonefritis membranoproliferativa. Reporte de caso / Clinical approach and diagnosis of membranoproliferative glomerulonephritis, case report

Resumen La glomerulonefritis membranoproliferativa es una lesión poco frecuente que abarca un grupo de patologías que comparten un patrón histológico en común y pueden originarse a partir de diversos mecanismos patogénicos. Mediante biopsias renales se ha establecido que en estas lesiones el daño renal inicial ocurre por el depósito de inmunoglobulinas que generan depósitos subendoteliales, subepiteliales y/o en la membrana basal glomerular, con lo cual el glomérulo adopta frecuentemente un aspecto lobulado. Se presenta el caso de un hombre de 48 años, quien ingresó al servicio de urgencias del Hospital de San José de Bogotá, Colombia, sin antecedentes relevantes y presentó proteinuria en rango nefrótico severo (62 gramos en 24 horas), elevación de nitrogenados y reporte de biopsia con glomerulonefritis membranoproliferativa. Se describe el enfoque clínico y diagnóstico de esta entidad.

Abstract The membranophroliferative glomerulonephritis is a rare lesion that covers a group of pathologies that share a histologic pattern, and can generate from diverse pathogenic mechanisms. Based on the findings of the kidney biopsy, starts a process to search the etiology of the lesion. The initial kidney damage occurs due to the deposit of immunoglobulins, complement elements or both in the mesangium and in the capillary endothelium, generating sub endothelial, sub epithelial, and/or glomerular basal membrane deposits, frequently adopting glomerulus a lobed aspect. We present a case of a man 48 years old admitted in the emergency of the San Jose Hospital in Bogotá, without relevant pathological background that debuts with proteinuria in severe nephrotic range (62 grams in 24 hours), nitrogenous elevation and biopsy report with membranophroliferative glomerulonephritis, we describe de clinical and diagnostic approach of this entity.

Pathology of C3 Glomerulopathy

C3 glomerulopathy is a renal disorder involving dysregulation of alternative pathway complement activation. In most instances, a membranoproliferative pattern of glomerular injury with a prevalence of C3 deposition is observed by immunofluorescence microscopy. Dense deposit disease (DDD) and C3 glomerulonephritis (C3GN) are subclasses of C3 glomerulopathy that are distinguishable by electron microscopy. Highly electron-dense transformation of glomerular basement membrane is characteristic of DDD. C3GN should be differentiated from post-infectious glomerulonephritis and other immune complex-mediated glomerulonephritides showing C3 deposits.

Rare Occurrence of Type lll Membranoproliferative Glomerulonephritis in a patient with Hepatitis C in Sustained Virologic Response Phase

@#Hepatitis C virus (HCV) infection is known to cause acute and chronic active hepatitis. In addition, HCV also has systemic disorder involvement that links to various extra-hepatic complications. We report a case of a patient which has been diagnosed to have Hepatitis C Genotype 3A who has been started on antiviral. He achieved end treatment response and sustained virologic response. During routine follow up, he experienced acute kidney injury. Renal biopsy showed type III membranoproliferative glomerulonephritis. His proteinuria improved greatly with the addition of angiotensin converting enzyme inhibitor. This case highlights the possibility of appearance of HCV related glomerulonephritis in patient who has sustained virological response.

Respuesta a la farmacoterapia en pacientes con glomerulonefritis membranoproliferativa en una clínica de Barranquilla, Colombia. 2007-2014 / Response to pharmacotherapy in patients with membranoproliferative glomerulonephritis in a hospital of Barranquilla, Colombia, between 2007 and 2014

Resumen Introducción. La glomerulonefritis membranoproliferativa (GnMP) es un patrón de lesión glomerular hipercelular mesangial con adelgazamiento de la membrana basal glomerular y proliferación endocapilar que está mediado por las inmunoglobulinas o el sistema del complemento en el mesangio y endotelio capilar. Objetivo. Evaluar la respuesta a la farmacoterapia en pacientes diagnosticados con GnMP en una clínica de Barranquilla entre los años 2007 y 2014. Materiales y métodos. Estudio de cohorte retrospectivo en el que se evaluaron 58 pacientes con diagnóstico de GnMP por biopsia renal, quienes se clasificaron como respondedores y no respondedores. Se realizó una evaluación de tratamiento estándar según tipo de GnMP: mediado por complemento y mediado por inmunocomplejos e inmunofluorescencia negativa a los 6 y 12 meses de tratamiento. Resultados. La edad promedio de los participantes fue de 35±13 años. De 58 pacientes, 52% eran mujeres, 63% desarrolló enfermedad renal crónica (ERC) al año de evaluación, 25.8% logró remisión (22.4% completa y 3.4% parcial) y 74.2% no logró entrar en remisión. Conclusión. La GnMP es una causa importante de ERC entre la población estudiada. La respuesta al tratamiento inmunosupresor no demostró beneficios estadísticamente significativos, independiente del tipo de GnMP.

Abstract Introduction: Membranoproliferative glomerulonephritis (MPGN) is a pattern of mesangial hypercellular glomerular lesion with thinning of the glomerular basement membrane and endocapillary proliferation, mediated by immunoglobulin and the complement system of the mesangium and capillary endothelium. Objective: To assess the response to pharmacotherapy in patients diagnosed with MPGN in a hospital of Barranquilla between 2007 and 2014. Materials and methods: Retrospective cohort study in which 58 patients diagnosed with MPGN by renal biopsy were assessed and classified as responsive and non-responsive. A standard treatment assessment was performed according to the type of MPGN: mediated by the complement system, mediated by immunocomplexes, and negative immunofluorescence at 6 and 12 months of treatment. Results: The average age of the participants was 35±13 years. Of 58 patients, 52% were female, 63% developed chronic kidney disease (CKD) one year after the assessment, 25.8% achieved remission (22.4% complete and 3.4% partial) and 74.2% failed to enter remission. Conclusion: MPGN is one of the most important causes of CKD among the studied population. Response to immunosuppressant treatment showed no statistically significant benefits, regardless of the type of MPGN.

Membranoproliferative glomerulonephritis in a patient with chronic brucellosis

Brucellosis is the most common zoonotic disease in Greece, with an endemic distribution and can affect any organ. Infiltration of the renal parenchyma causes acute and chronic interstitial nephritis with granulomas, whereas renal glomeruli are rarely affected. The disease has been sporadically reported, and it causes various histopathologic patterns. Herein, we describe the case of a 39-year-old stock breeder with a history of recurrent episodes of bacteremia caused by Brucella melitensis over a period of 3 years. Two months after the last episode of bacteremia, he presented with mild renal insufficiency, nephrotic range proteinuria, and microscopic hematuria. A renal biopsy revealed membranoproliferative glomerulonephritis with a pattern of focal-segmental nodular sclerosis and moderate tubulointerstitial fibrosis. The patient received antimicrobial and corticosteroid therapy with partial remission of the nephrotic syndrome.

Crioglobulinemia: relação entre hepatite C e glomerulonefrite / Cryoglobulinemia: link between hepatitis C and glomerulonephritis

A Hepatite C é um problema de saúde mundial. Uma associação entre infecção pelo vírus da hepatite C (HCV) e crioglobulinemia mista com doença renal tem sido descrita, sendo a glomerulonefrite membranopoliferativa (GNMP) tipo I o acometimento renal mais comum. A GNMP é frequentemente associada com a crioglobulinemia mista tipo II. Esse relato de caso objetiva descrever fatores clínicos dos pacientes com crioglobulinemia mista, a qual é uma manifestação extra-hepática da infecção por HCV, assim como discutir a sua fisiopatologia e tratamento, baseado no relato de caso. (AU)

Hepatitis C is a public health concern worldwide. An association of hepatitis C virus (HCV) infection with mixed cryoglobulinemia and renal disease has been described, and type I membranoproliferative glomerulonephritis (MPGN) is the most common. MPGN is often associated with type II mixed cryoglobulinemia. This case report aimed to describe the clinical features of patients with mixed cryoglobulinemia, a major extrahepatic manifestation of HCV infection, and to discuss its pathophysiology and treatment of HCV infection based on the case report. (AU)

Clinical and histological features of patients with membranoproliferative glomerulonephritis classified by immunofluorescence findings / Características clínicas e histológicas de pacientes com glomerulonefrite membranoproliferativa classificados por achados de imunofluorescência

Abstract Background: New classification for membranoproliferative glomerulonephritis has been proposed in the literature. The aim of this study was to compare the clinical, biochemical, etiology and renal biopsy findings of these patients grouped by immunofluorescence as proposed by the new classification. Methods: Patients with renal biopsy-proven membranoproliferative glomerulonephritis unrelated to systemic lupus erythematosus, diagnosed between 1999 and 2014. The patients were divided according to immunofluorescence: Immunoglobulin positive group, C3 positive only and negative immunofluorescence group. Results: We evaluated 92 patients, the majority of which were in the immunoglobulin positive group. Infectious diseases, hepatitis C virus and schistosomiasis, were the most frequent etiology. A negative immunofluorescence group had more vascular involvement in renal biopsy compare with others groups. Conclusions: The only difference between the groups was higher vascular involvement in renal biopsy in negative immunofluorescence group. These new classification was satisfactory for the finding of etiology in one part of the cases.

Resumo Introdução: Uma nova classificação para glomerulonefrite membranoproliferativa foi proposta na literatura. O objetivo deste estudo foi comparar os achados clínicos, bioquímicos, etiológicos e da biópsia renal desses pacientes agrupados por imunofluorescência, conforme proposto pela nova classificação. Métodos: Pacientes com glomerulonefrite membranoproliferativa comprovada por biópsia renal, não relacionada ao lúpus eritematoso sistêmico, diagnosticados entre 1999 e 2014. Os pacientes foram divididos de acordo com a imunofluorescência: grupo positivo por imunoglobulina, grupo positivo por C3 apenas e grupo com imunofluorescência negativa. Resultados: avaliamos 92 pacientes, a maioria dos quais estava no grupo de imunoglobulina positiva. Doenças infecciosas, o vírus da hepatite C e a esquistossomose, foram as etiologias mais frequentes. Um grupo com imunofluorescência negativa apresentou maior comprometimento vascular na biópsia renal quando comparado com os outros grupos. Conclusões: a única diferença entre os grupos foi o maior envolvimento vascular na biópsia renal no grupo de imunofluorescência negativa. Esta nova classificação foi satisfatória para a descoberta de etiologia em uma parte dos casos.

Síndrome nefrótico secundario a glomerulonefritis membranoproliferativa por síndrome de Sjögren / Nephrotic syndrome secondary to membranoproliferative glomerulonephritis due to Sjögren's syndrome

Resumen El síndrome de Sjögren (SS), enfermedad autoinmune crónica, primaria o secundaria a otros desórdenes inmunológicos. La prevalencia es 0.05-0.23%, relación hombre: mujer 1:9, grupo etáreo 52-72 años. La afectación renal en el SSP (primario) puede ocasionar compromiso glomerular y/o tubular. Reportamos el caso de un hombre de 56 años, previamente sano que presentó síndrome nefrótico en el contexto de una glomerulonefritis membranoproliferativa (GMNMP), se diagnosticó SS con compromiso glomerular y se realizó revisión de la literatura. (Acta Med Colomb 2017; 42: 136-139).

Abstract Sjögren's syndrome (SS) is a chronic autoimmune disease primary or secondary to other immunological disorders. The prevalence is 0.05-0.23%, male: female ratio 1:9, age group 52-72 years. Renal involvement in primary SS can lead to glomerular and/or tubular involvement. The case of a 56-year-old man previously healthy that presented nephrotic syndrome in the context of a membranoproliferative glomerulonephritis (MPGN) and was diagnosed with SS with glomerular involvement is reported, and a literature review was performed. (Acta Med Colomb 2017; 42: 136-139).

Evolución al año de la nefritis lúpica con proliferación extra capilar focal / Progression of focal lupus nephritis with extracapillary proliferation after one year

INTRODUCCIÓN: La nefritis lúpica proliferativa (NPL) con proliferación extra capilar focal (PECF) es una entidad frecuente en las biopsias renales, pero poco se conoce acerca de su evolución. Este estudio se realizó para investigar la correlación clínica, histológica y de laboratorio en la evolución de estos pacientes. OBJETIVO: Analizar las biopsias de nefritis lúpicas proliferativas focales o difusas (clases III y IV de la OMS), y su evolución de acuerdo al grado de proliferación extracapilar en un período de 10 años, en el Hospital de Clínicas "José de San Martín". MATERIAL Y MÉTODOS: Estudio observacional retrospectivo, se incluyeron 147 pacientes con GN clase III o IV, estudiados en un período de 10 años. Se subdividieron en base al compromiso extracapilar en: grupo 1 (5- <25% de semilunas, n=33), grupo 2 (25- 50% semilunas, n=34) y grupo 3 (>50% semilunas, n= 20) o proliferación extracapilar difusa (PECD). Los pacientes con nefritis lúpica III y IV, sin compromiso extra capilar, se denominó grupo control (n=60); las características clínicas, laboratorios y evolución en cuanto alcanzar remisión completa al año fueron comparadas entre los subgrupos y el grupo de control. Remisión completa se definió según normas KDIGO 2012. Se realizó un análisis univariado y posteriormente un multivariado, ambos con regresión logística. RESULTADOS: Hubo 124 mujeres (84,35%) y 23 hombres (15,65%) con una edad media de 35±10 años. Edad media en el grupo control, 32±10 años. El univariado mostro mayor remisión completa en el grupo control con odds ratio 0.05 (0.02.-0.12) p=0.001, y menor remisión completa en los 3 subgrupos con proliferación extra capilar, inclusive el grupo I con un odds ratio 7.61 (2,42-23.9) y valor de p=0.001 en dicho grupo. El análisis multivariado evidenció que la persistencia de la proteinuria a los 6 meses tenía significación estadística para no alcanzar la remisión completa, con un odd ratio 2.81 (1.58-5.0), valor de p=0.001. CONCLUSIÓN: La presencia de distintos grados de semilunas en la histología de las NL proliferativas (NPL), clase III y IV, y la persistencia de la proteinuria a los 6 meses, son marcadores independiente para no alcanzar la remisión completa al año

IINTRODUCTION: Although focal lupus nephritis (FLN) with extracapillary proliferation (ECP) is usually found in renal biopsies, little is known about its course. This study was carried out to investigate the correlation of clinical, histological and laboratory findings in these patients' progress. OBJECTIVE: To analyze focal or diffuse proliferative lupus nephritis biopsies (class III and IV, according to WHO) and their development regarding the level of extracapillary proliferation throughout a period of 10 years at Hospital de Clínicas 'José de San Martín'. METHODS: In this retrospective observational study, 147 patients with class III or IV GN were observed for 10 years. According to the degree of extracapillary proliferation, they were divided into the following groups: group 1 (5% <25% of crescents, n=33), group 2 (25% 50% of crescents, n=34) and group 3 [>50% of crescents, i.e. diffuse proliferative nephritis (DPN), n=20]. Patients suffering from class III and IV lupus nephritis with no extracapillary proliferation formed the control group (n=60). After one year, clinical and laboratory findings as well as the development of the condition among the four groups were compared to assess their level of remission. Complete remission was considered as defined in KDIGO 2012 Guideline. A univariate logistic regression analysis and a subsequent multivariate one were performed. RESULTS: There were 124 women (84.35%) and 23 men (15.65%) with an average age of 35±10. The average age in the control group was 32±10. The univariate analysis showed a greater number of patients who achieved complete remission in the control group with an odds ratio of 0.05 (0.02 0.12) p=0.001, whereas in the 3 groups with extracapillary proliferation fewer patients reached this state, even in group 1, which had an odds ratio of 7.61 (2.42 23.9) and p=0.001. The multivariate analysis revealed that the persistent protein in the urine after 6 months was statistically significant for not achieving complete remission, the odds ratio being 2.81 (1.58 5.0) and p=0.001. CONCLUSION: The presence of various degrees of crescent formation in class III and IV proliferative lupus nephritis (PLN) shown in the histological findings and the persistent protein in the urine at 6 months constitute independent factors for not achieving complete remission after one year

Research advances of membranoproliferative glomerulonephritis in children / 国际儿科学杂志

Membranoproliferative glomerulonephritis(MPGN)is immune pathological diagnosis,and it has all sorts of clinical manifestations,poor prognosis and high case fatality rate.The latest classification of MPGN is divided into immunecomplex-mediated and complement-mediated.On the basis of the potential patho-physiology,its related causes can be assessed in order to choose a more appropriate treatment method.Long alter-nate day glucocorticoid-therapy is beneficial to idiopathic MPGN in children.Biological agents used in the treat-ment of MPGN has made great progresses.

Analysis of clinical and pathological relationship of idiopathic membranous nephropathy in elderly patients / 中华老年医学杂志

Objective To understand the clinical and pathological characteristics of idiopathic membranous nephropathy,and to investigate their relationships in the elderly patients.Methods The clinical and pathological data of idiopathic membranous nephropathy diagnosed by renal biopsy in the recently 10 years in our hospital were retrospectively analyzed and compared.Results Sex composition variable was not significant between elderly and non-elderly groups,with male accounting for 57.4％ and 59.2％ respectively (P＞0.05).The ratio of idiopathic membranous nephropathy in primary glomerular disease was higher in the elderly than in non-elderly group (42.8％ vs.17.4％,x2 =119.058,P＜0.01).The course of the disease,the occurrence of edema,hypertension,levels of blood urea nitrogen,serum creatinine,blood IgG,IgA and IgG/IgM ratio were higher,while glomerular filtration rate and blood IgM level were lower in elderly group than in non-elderly group (all P＜0.05).The risk for membranous nephropathy was higher in elderly than in non-elderly group (Z=-5.853,P＜0.01).Nephrotic syndrome was common in the clinical diagnosis in the two groups,but it was more common in elderly group than in non-elderly group (x2 =6.825,P=0.033).Membranous nephropathy stage Ⅱ was common in the two groups,and there were no significant differences in the pathological stages,clinical manifestations and their relationships between the two groups (all P＞0.05).Conclusions The risk for idiopathic membranous nephropathy is higher in elderly group than in non-elderly group.Edema,hypertension and renal dysfunction occur more commonly in elderly patients with idiopathic membranous nephropathy.

Linfoma no Hodgkin de células del manto con infiltración renal y glomerulopatía mesangiocapilar asociada / Mantle cell non-Hodgkin lymphoma with renal infiltration and associated mesangiocapillary glomerulopathy

El linfoma de células del manto es una forma infrecuente de linfoma no Hodgkin con alta frecuencia de recaídas y peor pronóstico. Los linfomas pueden inducir daño renal de diferentes modos, entre los que se encuentran la infiltración renal y el desarrollo de glomerulopatías. Se presentó un caso en el que se asocia el comienzo clínico de un linfoma de células del manto con infiltración parenquimatosa renal y glomerulonefritis mesangiocapilar sin crioglobulinemia. Este constituye el primer caso reportado con esta asociación y pone en evidencia las diferentes formas de afectación renal del linfoma no Hodgkin y el valor del estudio histológico renal, para el diagnóstico y el pronóstico de las enfermedades oncohematológicas con daño renal

Mantle cell lymphoma is an infrequent type of non-Hodgkin lymphoma with high relapse rates and poor prognosis. Lymphomas can induce kidney damage in several ways, including renal infiltration and the development of glomerulopathies. A case associated to the clinical onset of a mantle cell lymphoma with lymphocytic infiltration into the renal parenchyma and mesangiocapillary glomerulonephritis without cryoglobulinemia was presented. This is the first case report that describes this association which evidences the different types of renal lesions in no Hodgkin lymphoma and the value of renal histological study for the diagnosis and prognosis of onco-hematologic diseases with kidney damage

A Case of Membranoproliferative Glomerulonephritis in a Patient with Type 2 Diabetes Mellitus / 영남의대학술지

Diabetic nephropathy (DN) is a common complication and the leading cause of end-stage renal disease (ESRD) in diabetic patients. The occurrence of non-diabetic renal disease (NDRD) in diabetic patients has been increasingly recognized in recent years. Generally, renal injuries in DN are deemed difficult to reverse, whereas some NDRDs are often treatable and even remittable. Thus, the diagnosis of NDRD in patients with diabetes mellitus (DM) via a kidney biopsy would be significant for its prognosis and therapeutic strategy. According to recent studies, the most common NDRD is IgA nephropathy in type 2 diabetic patients, and some cases of minimal change disease and membranous glomerulonephritis have been reported in Korea. However, membranoproliferative glomerulonephritis (MPGN) is an uncommon condition in diabetic patients. To our knowledge, there has been no case yet of MPGN, except in a child with type 1 DM. We present an unusual case of a 27-year-old woman who had type 2 DM with MPGN, as confirmed via a kidney biopsy.

A case of membranoproliferative glomerulonephritis associated with metastatic colon cancer

No abstract available.

Primary glomerulonephritis: A review of important recent discoveries

The publication of the Kidney Disease: Improving Global Outcomes (KDIGO) guidelines on the treatment of glomerular diseases in 2012 marked a milestone in this field, asitisthe first time that comprehensive guidelines are provided for such disease entities. The current review focuses on major findings, both path ogenesis related and clinical, in the primary glomerulonephritis that have been made after the guidelines came into effect.

Dense Deposit Disease in Korean Children: A Multicenter Clinicopathologic Study

The purpose of this study was to investigate the clinical, laboratory, and pathologic characteristics of dense deposit disease (DDD) in Korean children and to determine whether these characteristics differ between Korean and American children with DDD. In 2010, we sent a structured protocol about DDD to pediatric nephrologists throughout Korea. The data collected were compared with previously published data on 14 American children with DDD. Korean children had lower 24-hr urine protein excretion and higher serum albumin levels than American children. The light microscopic findings revealed that a higher percentage of Korean children had membranoproliferative glomerulonephritis patterns (Korean, 77.8%; American, 28.6%, P = 0.036), whereas a higher percentage of American children had crescents (Korean, 0%; American, 78.6%, P < 0.001). The findings from the electron microscopy revealed that Korean children were more likely to have segmental electron dense deposits in the lamina densa of the glomerular basement membrane (Korean, 100%; American, 28.6%, P = 0.002); mesangial deposit was more frequent in American children (Korean, 66.7%; American, 100%, P = 0.047). The histological findings revealed that Korean children with DDD were more likely to show membranoproliferative glomerulonephritis patterns than American children. The degree of proteinuria and hypoalbuminemia was milder in Korean children than American children.

A Case of Nutcracker Syndrome Combined with Idiopathic Membranoproliferative Glomerulonephritis, Type I / 대한내과학회지

The Nutcracker syndrome refers to compression of the left renal vein between the aorta and superior mesenteric artery, which results in renal vein and left gonadal vein varices, hematuria, and left flank pain. Here, we report a case of biopsy-proven membranoproliferative glomerulonephritis with radiologically documented Nutcracker syndrome. The patient presented with generalized edema for several months. In order to evaluate isomorphic hematuria, we performed computed tomography (CT), which showed Nutcracker syndrome. A renal biopsy revealedmembranoproliferative glomerulonephritis, Type I. In conclusion, inadults with Nutcracker syndrome that involves severe proteinuria, the possibility of combined glomerulonephritis must be considered.

A Case of Nutcracker Syndrome Combined with Idiopathic Membranoproliferative Glomerulonephritis, Type I / 대한내과학회지

The Nutcracker syndrome refers to compression of the left renal vein between the aorta and superior mesenteric artery, which results in renal vein and left gonadal vein varices, hematuria, and left flank pain. Here, we report a case of biopsy-proven membranoproliferative glomerulonephritis with radiologically documented Nutcracker syndrome. The patient presented with generalized edema for several months. In order to evaluate isomorphic hematuria, we performed computed tomography (CT), which showed Nutcracker syndrome. A renal biopsy revealedmembranoproliferative glomerulonephritis, Type I. In conclusion, inadults with Nutcracker syndrome that involves severe proteinuria, the possibility of combined glomerulonephritis must be considered.