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.

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).

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.

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

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.

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.

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.

Membranoproliferative Glomerulonephritis Associated with Papillary Thyroid Carcinoma / 대한신장학회지

Malignant tumors have been shown to be a major secondary cause of nephrotic syndrome. They have been associated with different glomerulopathy depending on their type. Membranoproliferative glomerulonephritis (MPGN) rarely develops in solid tumors, although cases have been reported in renal cell cancer, melanoma, lung cancer, and tumors in the urogenital system. However, to our knowledge, there have been no case reported of MPGN associated with thyroid cancer. In the present case, we observed MPGN associated with thyroid cancer in a 44-year-old woman with nephrotic syndrome and renal insufficiency. Her thyroid ultrasound revealed a tumor measuring 1.01x1.14x1.48 cm with an indistinct border that was partially calcified. The tumor was confirmed to be papillary carcinoma by percutaneous needle aspiration biopsy. Renal biopsy showed chronic tubulointerstitial nephritis and MPGN. On day 45 of admission, total thyroidectomy and neck lymphadenectomy were performed. After total thyroidectomy, serum total protein, albumin, C3, and C4 normalized. In conclusion, this case represents the first report of simultaneous development of MPGN and thyroid cancer.

Treatment of Posttransplantation Recurrent Glomerulonephritis: IgA Nephropathy, Membranous Nephropathy, Membranoproliferative Glomerulonephritis / 대한이식학회지

As the clinical outcomes improve in kidney transplantation, largely because of the prevention of loss due to acute rejection, the incidence of allograft loss due to recurrent glomerulonephritis have become more important. Actually recurrent glomerulonephritis is the third most common cause of graft failure, ranking only behind immunologic rejection and death with a functioning graft. Preventive and therapeutic treatment strategies are necessary for those patients at risk. Current newer immunosuppressive protocols over the past 10 years have not affected the rate of disease recurrence or graft loss. In a certain recurrent glomerulitis, for example membranous nephropathy, there is emerging evidence that rituximab may be efficacious; however, larger clinical trials are warranted.

Proliferative Glomerulopathy with Unusual Subendothelial Deposits of Striated Structure in Multiple Myeloma / 대한신장학회지

A 50-year-old woman was admitted for the evaluation of proteinuria and renal biopsy. On the basis of the serum monoclonal protein, marrow plasma cell dyscrasia and end organ damage (nephrotic range proteinuria), multiple myeloma was diagnosed. A renal biopsy showed a membranoproliferative glomerulonephritis pattern of injury and unusual organized deposits of striated structure in the subendothelial space, which were identified as non-amyloid non-immunoglobulin-derived deposits. These deposits contained regularly stacked straight electron-dense bands, which have not been described in the setting of paraproteinemia and/or plasma cell dyscrasia.

Microscopic Polyangiitis Presenting as Membranoproliferative Glomerulonephritis / 대한류마티스학회지

Microscopic polyangiitis (MPA) is characterized by pauci-immune necrotizing small vessel vasculitis without clinical or pathological evidence of necrotizing granulomatous inflammation. The kidney is the most often affected organ in the majority of patients with MPA, and renal manifestations are usually the first symptoms. Glomerular capillaries are affected most often, resulting in necrotizing glomerulonephritis, usually in a crescent formation, with no or few immune deposits able to be demonstrated at the sites of vasculitis and glomerulonephritis. We report a case of microscopic polyangiitis in both legs with pitting edema in a 50-year-old female. Laboratory findings showed hematuria, proteinuria, and a positive peripheral antineutrophil cytoplasmic antibody. A renal biopsy revealed pauci-immune splitting and necrotizing capillary loop walls necrotizing vasculitis and membranoproliferative glomerulonephritis (MPGN). With a diagnosis of MPA, she has been managed with high dose steroid and cyclophosphamide. To our knowledge, this is the first reported case of MPA with MPGN.

A Case of Membranoproliferative Glomerulonephritis with False-Positive Reaction for Anti-GBM Antibody / 대한신장학회지

Anti-glomerular basement membrane (Anti-GBM) nephritis is an autoimmune disorder characterized by rapidly progressive crescentic glomerulonephritis (RPGN). The treatment of anti-GBM nephritis with plasmapheresis, steroids and immunosuppressant has improved outcomes. An early diagnosis is essential for the survival of patients and a recovery of renal function. The diagnosis of anti-GBM disease has been traditionally based on the demonstration of linear deposits of immunoglobulins along the glomerular basement membrane by immunofluorescence (IF) microscopy. However, a kidney biopsy cannot always be easily performed in such ill patients. Recent development of specific enzyme immunoassays for anti-GBM antibody in the serum has made possible a provisional diagnosis without a kidney biopsy. A 46-year-old male patient with hypertension and hepatitis B presented with generalized edema and general weakness. Laboratory findings were compatible with acute renal failure and nephrotic syndrome with positive serum anti-GBM antibodies. After plasmapheresis with steroid pulse therapy, renal biopsy was performed and diagnosed as membranoproliferative glomerulonephritis (MPGN) with granular deposit of Ig G and C3. Follow-up antibody titers were negative. This case demonstrates the possibility of false-positive anti-GBM antibody in the serum. Therefore, enzyme immunoassay for anti-GBM antibody should be used only as a screening or follow-up test in patients that have been confirmed positive by IF microscopy.

A Case of IgA Nephropathy with a Membranoproliferative Glomerulonephritis-like Pattern Presenting as Massive Ascites in a Patient with Alcoholic Liver Disease / 대한신장학회지

IgA nephropathy can occur commonly in alcoholic liver cirrhosis and is the most common form of secondary IgA nephropathy. Defective clearance of IgA-containing complexes by liver is thought to contribute to the development of IgA nephropathy in alcoholic cirrhosis. Although IgA deposition is found up to 64% in autopsy cases of alcoholic cirrhosis, most patients have mild clinical symptoms, and nephrotic range proteinuria occurs rarely in these patients. We report a case of IgA nephropathy with a membranoproliferative pattern that is detected with unusual massive ascites in a patient with alcoholic liver disease. A 60-year-old male patient was referred to our hospital for evaluation and management of abdominal distension. Abdominal ultrasonographic findings were compatible with diffuse liver cirrhosis with splenomegaly and large amount of ascites. He had nephrotic range proteinuria, azotemia, hyperlipidemia, and hematuria in dipstick. Renal biopsy performed under the impression of acute nephritis revealed mesangial and endocapillary proliferative glomerulonephritis with double contour of capillary loop. Immunofluorescence findings showed mesangial IgA and C3 deposit, compatible with IgA nephropathy. He was treated with high dose steroid, and steroid was tapered during 2 months. Steroid treatment induced complete remission state, and ascites was resolved.