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.

Vasculitis crioglobulinémica con compromiso renal y síndrome de Sjögren / Cryoglobulinemic vasculitis with renal involment and Sjögren's syndrome

La vasculitis crioglobulinémica es una vasculitis de vasos pequeños que se da en presencia de crioglobulinas séricas. Las crioglobulinemias mixtas son las más frecuentes y se asocian a infecciones crónicas, típicamente al virus de la hepatitis C, así como a enfermedades linfoproliferativas y autoinmunes, más a menudo al síndrome de Sjögren. Las manifestaciones clínicas incluyen púrpura de los miembros inferiores, neuropatía periférica, artralgias y glomerulonefritis. La presencia de vasculitis crioglobulinémica en el contexto de un síndrome de Sjögren es marcadora de peor pronóstico. Presentamos el caso de una paciente con vasculitis crioglobulinémica con compromiso cutáneo y renal, que condujo al diagnóstico de un síndrome de Sjögren primario. (AU)

Cryoglobulinemic vasculitis is a small-sized vasculitis that occurs in the context of serum cryoglobulins. Mixed cryoglobulinemias are the most frequent and are associated with chronic infections, typically hepatitis C and autoimmune diseases, most commonly Sjögren's syndrome. Clinical manifestations include purpura of lower limbs, peripheral neuropathy, arthralgias and glomerulonephritis. The presence of cryoglobulinemic vasculitis in the context of Sjögren's syndrome is a marker of poor prognosis. We present the case of a patient with cryoglobulinemic vasculitis associated to cutaneous and renal involvement that led us to the diagnosis of primary Sjögren's syndrome. (AU)

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.

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)

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.

The Role of Thymic Stromal Lymphopoietin (TSLP) in Glomerulonephritis

Thymic stromal lymphopoietin (TSLP) is an interleukin-7-like cytokine that is an important trigger and initiator of many allergic diseases. TSLP promotes a T-helper type 2 (Th2) cytokine response that can be pathological. A relationship is formed both at the induction phase of the Th2 response through polarization of dendritic cells to drive Th2 cell differentiation and at the effector phase of the response, by promoting the expansion of activated T cells and their secretion of Th2 cytokines and TSLP. In transgenic mice with TSLP overexpression, it has been reported that TSLP leads to the development of mixed cryoglobulinemic membranoproliferative glomerulonephritis. In addition, TSLP can play an important role in the pathogenesis of IgA nephropathy and systemic lupus erythematosus-related nephritis. From our knowledge of the role of TSLP in the kidney, further studies including the discovery of new therapies need to be considered based on the relationship between TSLP and glomerulonephritis.

Bortezomib Treatment for Refractory Antibody-Mediated Rejection Superimposed with BK Virus-Associated Nephropathy during the Progression of Recurrent C3 Glomerulonephritis / 대한이식학회지

A 38-year-old man, who underwent a second kidney transplantation (KT), was admitted because of antibody-mediated rejection (AMR) complicated by BK virus-associated nephropathy (BKVAN). He was placed on hemodialysis at the age of 24 years because of membranoproliferative glomerulonephritis. At the age of 28 years, he underwent a living donor KT from his father; however, 1 year after the transplantation, he developed a recurrence of the primary glomerular disease, resulting in graft failure 2 years after the first KT. Ten years later, he received a deceased-donor kidney with a B-cell-positive-cross-match. He received 600 mg of rituximab before the KT with three cycles of plasmapheresis and immunoglobulin (0.5 g/kg) therapy after KT. During the follow-up, the first and second allograft biopsies at 4 and 10 months after KT revealed AMR with a recurrence of primary glomerular disease that was reclassified as C3 glomerulonephritis (C3GN). He received a steroid pulse, rituximab, plasmapheresis, and immunoglobulin therapies. The third allograft biopsy demonstrated that the BKVAN was complicated with AMR and C3GN. As the azotemia did not improve after repeated conventional therapies for AMR, one cycle of bortezomib (1.3 mg/m²×4 doses) was administered. The allograft function stabilized, and BK viremia became undetectable after 6 months. The present case suggests that bortezomib therapy may be applicable to patients with refractory AMR, even in cases complicated with BKVAN.

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).

Immune complex membranoproliferative glomerulonephritis associated with transjugular intrahepatic portosystemic shunts in alcoholic cirrhosis: two case reports

Objective: To report on 2 patients with alcoholic cirrhosis who were treated with transjugular intrahepatic portosystemic shunt [TIPS] placement

Clinical Presentation and Intervention: The 2 patients had a history of alcoholic cirrhosis, and TIPS surgery was performed on them. In both cases, 4 months after TIPS placement, proteinuria was observed along with histological alterations characteristic of immune complex membranoproliferative glomerulonephritis [MPGN]

Conclusion: The TIPS in one patient was successful without Immediate complications, while the other patient was referred for a combined liver-kidney transplant. In both cases, immune complex MPGN might have developed after TIPS placement probably due to a reduced immune complex

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

Shenhua Tablet inhibits mesangial cell proliferation in rats with chronic anti-Thy-1 nephritis

BACKGROUND: In China, mesangial proliferative glomerulonephritis (MsPGN) is one of the most common kidney diseases. In this study, we treated a rat model of chronic anti-Thy-1 MsPGN with Shenhua Tablet and evaluated whether the tablet was able to protect the kidney function. Thirty-six Wistar rats were randomly divided into six groups: (1) Sham surgery (Sham); (2) anti-Thy-1 nephritis model (Thy-1); (3) anti-Thy-1 nephritis model + irbesartan-treated (Irb); (4) anti-Thy-1 nephritis model + low-dose of Shenhua Tablet (SHL); (5) anti-Thy-1 nephritis model + medium-dose of Shenhua Tablet (SHM); (6) anti-Thy-1 nephritis model + high-dose of Shenhua Tablet (SHH). RESULTS: Thirteen weeks after drug treatment, urinary proteins were quantified and renal pathological changes were thoroughly examined at the time point of 24 h. Meanwhile, the expression levels of p-Erk1/2, cyclin D1 and p21 at the renal cortex were also tested. The levels of urinary proteins and total cholesterol in the blood were significantly reduced in rats treated with any drug tested in this study. The level of triglyceride was significantly reduced in all three Shenhua Tablet-treated groups. Renal pathomorphological scores were significantly improved in groups of Irb, SHM and SHH. Mesangial cell proliferation was significantly inhibited in any drug-treated group. p-Erk1/2 and cyclin D1 were downregulated whereas p21 was upregulated in the renal cortex. CONCLUSIONS: Our study indicated that Shenhua Tablet is able to inhibit the abnormal proliferation of mesangial cells and to prevent kidney damage, which is likely associated with downregulation of p-Erk1/2 and reduced activity of its downstream target-cyclin D1.

Complement regulation: physiology and disease relevance / 소아과

The complement system is part of the innate immune response and as such defends against invading pathogens, removes immune complexes and damaged self-cells, aids organ regeneration, confers neuroprotection, and engages with the adaptive immune response via T and B cells. Complement activation can either benefit or harm the host organism; thus, the complement system must maintain a balance between activation on foreign or modified self surfaces and inhibition on intact host cells. Complement regulators are essential for maintaining this balance and are classified as soluble regulators, such as factor H, and membrane-bound regulators. Defective complement regulators can damage the host cell and result in the accumulation of immunological debris. Moreover, defective regulators are associated with several autoimmune diseases such as atypical hemolytic uremic syndrome, dense deposit disease, age-related macular degeneration, and systemic lupus erythematosus. Therefore, understanding the molecular mechanisms by which the complement system is regulated is important for the development of novel therapies for complement-associated diseases.

A case of acquired partial lipodystrophy (Barraquer-Simons syndrome) in a 41-year-old Filipino woman

Acquired partial lipodystrophy, also known as Barraquer-Simons syndrome, is a rare disease characterized by progressive atrophy of adipose tissue primarily on the face, leading to a gaunt appearance. Usually manifesting in childhood and in women, the evolution of fat loss occurs in a cephalocaudad manner. The lower extremities are spared and may present with paradoxical hypertrophy. Common findings are C3 hypocomplementemia and positive C3 nephritic factor (C3Nef) but the pathogenesis remains unknown. The prognosis depends on presence of co-morbidities such as membranoproliferative glomerulonephritis. We report a 41-year-old woman who presented with a 23 year history of progressive fat atrophy on the face, trunk, and upper extremities with no skin changes or other symptoms. Subsequently, a three-year history of fat hypertrophy in both lower extremities was noted. Histopathologic findings and decreased C3 ultimately led to a diagnosis of acquired partial lipodystrophy. A thorough approach to this case is reported, along with a review of current literature on pathogenetic mechanisms suspected in the disease. Amongst the lipodystrophy syndromes, acquired partial lipodystrophy portends a relatively good prognosis. However, the cosmetic disfigurement and risk for membranoproliferative glomerulonephritis necessitate a multidisciplinary form of supportive management from a medical, surgical, and psychological point of view. With watchful supportive therapy, patients may lead normal, fulfilling lives.

New classification of membranoproliferative glomerulonephritis: a good start but a long way to go

No abstract available.

Reanalysis of membranoproliferative glomerulonephritis patients according to the new classification: a multicenter study

BACKGROUND: All types of membranoproliferative glomerulonephritis (MPGN) are progressive diseases with poor prognoses. Recently, a newly proposed classification of these diseases separated them into immune complex- and complement- mediated diseases. We investigated the frequency of C3 glomerulonephritis among previously diagnosed MPGN patients. METHODS: We conducted a retrospective study of patients diagnosed with MPGN at three tertiary care institutions between 2001 and 2010. We investigated the incidence of complement-mediated disease among patients diagnosed with MPGN. Progressive renal dysfunction was defined as a 50% reduction in the glomerular filtration rate or the need for renal replacement therapy. RESULTS: Among the 3,294 renal biopsy patients, 77 (2.3%) were diagnosed with MPGN; 31 cases were excluded, of which seven were diagnosed with systemic lupus nephritis, and the others were not followed for a minimum of 12 months after biopsy. Based on the new classification, complement-mediated MPGN was diagnosed in two patients (4.3%); only one patient developed progressive renal dysfunction. Among the immune complex-mediated MPGN patients, 17 patients developed progressive renal dysfunction. Serum albumin and creatinine levels at the time of MPGN diagnosis were risk factors of renal deterioration, after adjusting for low C3 levels and nephrotic syndrome. CONCLUSION: Complement-mediated glomerulonephritis was present in 4.3% of patients previously diagnosed with MPGN.

Significado de la ubicación de los depósitos de igA (mesangio capilares versus mesangiales puros) en la nefropatía igA y su asociación con las variables morfológicas de la clasificación de Oxford y varios datos demográficos / Significance of igA deposits location (mesangiocapillary versus pure mesangial) in igA nephropathy and its association with morphologic variables of Oxford classification and various demographic data

Introducción: La nefropatía IgA se caracteriza por la presencia de depósitos glomerulares con predominio de IgA. Dentro de la descripción de la nefropatía IgA, existe una variación en la ubicación de los depósitos de inmunoglobulina A, desde el área mesangial hasta las paredes capilares. Objetivo: El objetivo de este estudio es determinar la posible correlación entre la ubicación de los depósitos de IgA y las variables morfológicas de la clasificación de Oxford (MEST, por sus siglas en inglés). Proliferación (hipercelularidad) mesangial (M) y endocapilar (E), glomeruloesclerosis(S) y atrofia tubular y fibrosis intersticial (T), y diversos datos clínicos de pacientes con nefropatía por inmunoglobulina. Métodos: El diagnóstico patológico de la nefropatía IgA requiere la demostración de depósitos inmunes con predominio de IgA con un patrón mesangial o mesangiocapilar a través de la microscopia por inmunofluorescencia (IF por su sigla en ingles). Los depósitos inmunes fueron semicuantificados con grados de fluorescencia de 0 a 3 cruces (+). La definición de la nefropatía por IgA requiere la presencia de depósitos de IgA difusos y globales con ≥ 2 + de fluorescencia y la ausencia de depósitos de C I q. Todas las biopsias renales realizadas entre julio del 2009 y julio de 2012, fueron enviadas a nuestro laboratorio de patología renal para analizarlas. Ninguno de los pacientes fue tratado antes de habérsele realizado la biopsia. Las biopsias con menos de 8 glomérulos fueron excluidas del estudio. Ninguno de los pacientes recibió un diagnóstico de nefropatía IgA, si había antecedentes de enfermedad vascular del colágeno o cirrosis hepática en los cuestionarios clínicos, los análisis de laboratorio o en el historial médico obtenidos al ingresar a los pacientes para realizarles la biopsia renal. Resultados: Un total de 114 biopsias fueron incluidas en el estudio. La edad media de los pacientes fue de 37,7 ± 13,6 años. Los pacientes se dividieron en dos grupos: depósitos puros mesangiales y depósitos mesangiocapilares. El número medio de glomérulos obtenidos por biopsia fue de 14,8 ± 7,2. El nivel medio de la proteinuria fue de 1742 ± 1324 mg /día (mediana = 1500 mg /día). En todas las biopsias, el número mediode glomérulos totalmente esclerosados fue de 2,4 ± 2,9 (mediana =1 glomérulo). Asimismo, la media del nivel de creatinina sérica fue de 1,6 ± 1,5 /dl (mediana = 1,2 mg / dl). En este estudio, el 10,5 por ciento de las biopsias renales tenían depósito de IgA mesangiocapilares. No se encontró ninguna asociación significativa entre la proporción de glomérulos totalmente esclerosados, la proliferación extracapilar, el porcentaje de fibrosis peri-glomerular, el engrosamiento de la cápsula de Bowman, el porcentaje de fibrosis intersticial, la proliferación mesangial de cualquier grado, o el ensanchamiento mesangial con depósitos mesangiales puros o depósitos mesangiocapilares (p≤ 0,05). No hubo ninguna asociación significativa entre la edad, la creatinina sérica y los niveles de proteinuria con depósitos mesangiales puros o mesangiocapilares (p≥0,05). Entre las cuatro variables morfológicas de la clasificación MEST de Oxford, únicamente la variable E (proliferación endocapilar) tuvo asociación significativa con depósitos mesangiocapilares (p=0,04) Conclusiones: La asociación entre depósitos mesangiocapilares IgA y la proliferación endocapilar puede implicar una mayor gravedad de la enfermedad por nefropatía IgA. Por lo tanto, se recomienda que la ubicación y la intensidad de los depósitos de IgA se incluyan de forma sistemática en los informes de biopsia renal.

Introduction: IgA nephropathy is characterized by the presence of IgA-dominant glomerular deposits. Within this description, there is variation in the location of this immunoglobulin, from mesangial area to capillary walls. Objectives: The aim of this study is to determine the potential correlation between the location of IgA deposits and morphologic variables of Oxford classification (MEST) and various clinical data of patients with immunoglobulin A nephropathy (IgAN). Results: A total of 114 biopsies were enrolled to the study. Mean age of patients was 37.7 ± 13.6 years. Patients were divided into two groups of pure mesagnial and mesangiocapillary deposits. In this study 10.5 percent of renal biopsies had mesangial-capillary IgA deposits. There was not significant association of proportion of totally sclerosed glomeruli, extracapillary, proliferation, percentage of peri-glomerular fibrosis, thickening of the Bowman’s capsule, perent of interstitial fibrosis, mesangial proliferation in any degree and mesangial widening with pure mesangial or mesangial- capillary deposits (p>0.05).There was not significant association of age, serum creatinine and levels of proteinuria with pure mesangial or mesangiocapillary deposits (p>0.05).Among four morphologic variables of Oxford classification only E variable (endocapillary proliferation) had significant association with mesangiocapillary deposits (P=0.04). Conclusion: The association of mesangiocapillary IgA deposits with endocapillary proliferation may imply the severity of the disease. We recommend that the location and intensity of IgA is routinely included in the renal biopsy report.

Patterns of Glomerulonephritis in Uttarakhand State 13 Years Single Centre Experience at Teaching Institute.

Aims : Glomerular disease is considered to be a common cause of chronic kidney disease and its prevalence and histological pattern differ according to geographical area, race, age in different regions of the world. The present study was therefore conducted to study the pattern of different histological types of glomerulonephritis in this area of Uttarakhand region of India over an extended period of 13 years. Material and Methods : Retrospective study was conducted in the tertiary care centre of Uttarakhand state which included cases of different types of glomerulonephritis diagnosed on histopathology over a period of 13 years. Age, sex, clinical presentation, relevant investigations and the histopathological type of glomerulonephritis were noted for every case. Results : Histopathological examination due to various non-neoplastic renal diseases was done in total 218 cases and out of these glomerular diseases was diagnosed in 178 cases. Proteinurea and edema was the most common presentation. Primary glomerulonephritis was observed in 88.5% cases and secondary in 9.9%. Mesangioprolifrative glomerulonephritis was the most common primary glomerulonephritis and nephropathy due to amyloidosis was the most common type of secondary glomerulonephritis. Conclusion : The study concludes that primary glomerulonephritis is the most common renal disease with mesangioproliferative glomerulonephritis as the most common primary glomerulonephritis and amyloidosis nephropathy as the most common secondary glomerulonephritis which differs from other regions of world. The study may be useful to pathologists, nephrologists and health care providers to formulate a basic platform for effective diagnostic, therapeutic and research base for glomerular diseases so as to prevent its complications.

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