Rare Case of Bilateral Renal Vein Thrombosis: A Case Report

Bilateral renal vein thrombosis (RVT) is a rare clinical condition. Acase of a patient initially presenting with fever with a diagnosisof Plasmodium falciparum malaria was further investigated and it was found to have bilateral RVT. The patient was successfullytreated with anti-coagulants and immunosuppressant.

O papel da IgG4 na fisiopatogenia da nefropatia membranosa idiopática: estado da arte / The role of IgG4 in the physiopathology of idiopathic membranous nephropathy: state of the art

Nefropatia membranosa idiopática é uma causa de síndrome nefrótica cuja etiopatogenia não está completamente esclarecida. Trata-se de uma doença imunologicamente mediada, na qual a deposição de imunocomplexos decorre da reação antígenoanticorpo in situ, na região subepitelial glomerular. A maioria dos antígenos envolvidos identificados são alvos da IgG4, subclasse predominante em imunofluorescências renais na nefropatia membranosa idiopática, em contraste com as formas secundárias da doença, nas quais IgG1, IgG2 e IgG3 prevalecem. Apesar da IgG4 ser um subtipo de imunoglobulina com baixa capacidade de ativação do complemento, há várias evidências deste envolvimento na glomerulopatia (GMP). Esses dados, em conjunto com achados de depósitos glomerulares de lectina ligadora de manose, um dos principais componentes da via das lectinas do complemento, podem sugerir que tanto a via da lectina como a IgG4 estão envolvidas nesta patologia. Os motivos que desencadeiam a formação dos imunocomplexos e a ativação das vias do complemento nesta doença são incertos. A hipótese mais aceita é a de que a nefropatia membranosa idiopática resulte do conjunto de três condições: presença de proteínas com conformações alteradas que passam a atuar como autoantígenos, anticorpos do tipo IgG4 contra estes antígenos, e susceptibilidade genética. O objetivo foi verificar o possível papel da IgG4 na etiopatogenia da nefropatia membranosa primária segundo o que foi publicado até o momento na base de dados MEDLINE/PubMed, a partir de uma revisão narrativa.

Idiopathic membranous nephropathy is a frequent cause of nephrotic syndrome and its etiopathogenesis is not fully elucidated. In this immune mediated disease, the deposition of immune complexes is the result of an antigen-antibody reaction in situ, in the glomerular subepithelial region. Most of the antigens involved and so far identified are targets of IgG4, a predominant IgG subclass in renal immunofluorescence analysis of idiopathic membranous nephropathy, in contrast with secondary forms of the disease, in which IgG1, IgG2 and IgG3 are prevalent. Even though IgG4 is an immunoglobulin subclass with low complement activation capacity, there is abundant evidence of its involvement in the glomerulopathy. These data, together with findings of glomerular deposition of mannose-binding lectin ­ a major component of the lectin pathway in the complement system ­ may suggest that both the lectin pathway and IgG4 are involved in this pathology. The reasons behind the formation of immune complexes and the activation of complement pathways in this disease are unknown. The most widely accepted hypothesis is that idiopathic membranous nephropathy stems from a combination of three conditions: presence of proteins with altered conformations, which start to act as autoantigens; IgG4 antibodies against these antigens; and genetic susceptibility. The objective of this narrative review was to analyze the possible role of IgG4 in the etiopathogenesis of primary idiopathic membranous nephropathy based on articles published to date in the MEDLINE/PubMed database.

A Case of Bad Prognosis for Membranous Nephropathy in a Patient with Mixed Connective Tissue Disease

Incidence of renal involvement in mixed connective tissue disease (MCTD) is low. In the presence of glomerulonephritis, membranous nephropathy (MN) in MCTD is common. A 47-year-old woman presented with hypothyroidism. She developed Raynaud's phenomenon, arthralgia, and incomplete lupus erythematosus, diagnosed with MCTD. One year after then, the patient developed persistent proteinuria (1+) without hematuria. Following diagnosis with MCTD, her renal function began to deteriorate. The renal biopsy showed late stage MN. For the treatment of MN with mild proteinuria and MCTD, we prescribed an angiotensin II receptor blocker and 7.5 mg of methotrexate per week and 300 mg of hydroxychloroquine daily. The patient had a reduced estimated glomerular filtration rate of 55% for the subsequent eight years. The MN in MCTD is known to show good renal prognosis. Here, we report on a rare case of MN in MCTD in Korea with a bad prognosis.

Study on effect of leflunomide combined with prednisone in treating idiopathic membranous nephropathy / 中国基层医药

Objective To observe the effect of leflunomide and cyclophosphamide combined with prednisone in the treatment of idiopathic membranous nephropathy .Methods 30 patients with idiopathic membranous nephropa-thy were randomly divided into two groups ,15 cases in each group .A group was treated by leflunomide combined with prednisone , B group was treated by cyclophosphamide combined with prednisone , 6 months a course .The clinical effect,24h urine protein quantity,serum albumin levels,blood fat,renal functions and adverse reactions were com-pared.Results 3,6 months after treatment ,the remission rates in A group were 60.00%,73.33%,those in B group were 53.33%,66.67%(χ2 =0.965,0.896,all P>0.05).After treatment,24h urine protein quantity,serum albu-min levels in the two groups [A group:(1.33 ±1.25)g/24h,(38.24 ±4.84)g/L;B group:(1.42 ±1.37)g/24h, (37.12 ±5.43)g/L] were significantly lower than those before treatment [A group:(7.34 ±2.75)g/24h,(20.31 ± 7.33)g/L;B group:(7.22 ±2.84)g/24h,(20.46 ±7.73)g/L] (A group:t=6.232,5.734,all P0.05).There were no significant differences in above indicators between A group and B group after treatment (all P>0.05).The incidence rate of adverse reactions in A group (13.33%) was significantly lower than that in B group(40.00%)(χ2 =4.246,P<0.05).Conclusion The clinical efficacy of leflunomide is similar to cyclophosphamide in treating idiopathic membranous nephropathy with fewer adverse reactions .

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.

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.

Membranous glomerulonephritis in a patient with myelodysplastic syndrome-refractory cytopenia with multilineage dysplasia

A 74-year-old woman presented with edema in the lower extremities. Laboratory tests revealed anemia, thrombocytopenia, hypoalbuminemia, hypercholesterolemia, and nephrotic-range proteinuria. Myelodysplastic syndrome-refractory cytopenia with multilineage dysplasia (MDS-RCMD) was confirmed by bone marrow biopsy. Renal biopsy demonstrated membranous glomerulonephritis (MGN), stage I. Based on these clinicopathologic results, she was diagnosed as having MGN with MDS-RCMD. This is a rare case report of MGN in a parient with MDS-RCMD featuring nephrotic syndrome.

Sarcoidosis y compromiso renal: reporte de un caso y revisión de la literatura científica / Sarcoidosis and kidney disease: a case report and review of the literature

Se presenta el caso de una mujer de 30 años, que presentaba síntomas respiratorios y constitucionales de dos meses de evolución. Se documentó exudado linfocitario y proteinuria, y se descartó tuberculosis y neoplasia maligna. Se le practicó biopsia pleuraly de ganglio linfático, que reportaron sarcoidosis. Se tomó biopsia renal por presencia de proteinuria con pruebas serológicas negativas, y se encontró glomerulonefritis membranosa secundaria. Se inició tratamiento con prednisolona...

We describe the case of a 30 years old female with a two month history of respiratory symptoms and malaise. A lymphocytic pleural effusion and proteinuria were documented,tuberculosis and malignancy were ruled out. Biopsy of lymph nodes and pleura confirmed sarcoidosis. Due to non-nephrotic proteinuria with negative serology tests, kidney biopsy was performed, showing secondary membranous glomerulonephritis. Treatment with prednisolone was started...

Membranous Nephropathy in a 13-Year-Old Boy with Common Variable Immunodeficiency

Various forms of hypogammaglobulinemia can occur in patients with autoimmune diseases and vice versa. We report a 13-yr-old boy with membranous nephropathy and common variable immunodeficiency. He presented with the nephrotic syndrome, pneumonia with bronchiectasis, and profound hypogammaglobulinemia. Renal biopsy showed diffusely thickened glomerular capillary walls with 'spikes' suggesting a membranous nephropathy. Secondary causes were ruled out by laboratory studies; however, heavy proteinuria persisted with steroid therapy. Cyclosporine and intravenous immunoglobulin were added, and the patient was discharged with decreased proteinuria. Hypogammaglobulinemia may have a deleterious impact on the immune dysregulation in some patients with membranous nephropathy.

Experience of Anti-Viral Therapy in Hepatitis B-Associated Membranous Nephropathy, Including Lamivudine-Resistant Strains

BACKGROUND/AIMS: Chronic hepatitis B infection is a common cause of secondary membranous nephropathy (MN) in endemic areas. Lamivudine treatment improves renal outcome in patients with hepatitis B virus-associated MN (HBV-MN), but prolonged use leads to the emergence of lamivudine-resistant variants. We describe our experience treating lamivudine-resistant and other strains of HBV-MN with new antiviral drugs. METHODS: Of the 89 patients biopsied and diagnosed with MN from 1996 to 2011, 10 positive for hepatitis B surface antigen were recruited for this study. We investigated the clinical courses, therapeutic responses, and prognoses of patients with HBV-MN. RESULTS: The incidence of HBV-MN among the original 89 patients was 11.2%. Of these patients, four were treated with supportive care and six with antiviral drugs. One of the four patients treated with supportive care had a spontaneous remission. Four of the six patients treated with antiviral drugs were given lamivudine, and the other two were given entecavir. Two of the four patients treated with lamivudine achieved complete remission with seroconversion (i.e., development of anti-hepatitis B e antigen antibodies), whereas the other two had lamivudine-resistant strains, which were detected at 22 and 23 months after lamivudine treatment, respectively. We added adefovir to the treatment regimen for one of these patients, and for the other patient we substituted clevudine for lamivudine. Both of these patients experienced complete remission, as did the two patients initially treated with entecavir, neither of whom showed resistance to the drug. CONCLUSIONS: New nucleoside analogues, such as entecavir, adefovir, and clevudine, can be effective for treatment of HBV-MN, including lamivudine-resistant strains.

A Case of Basal Cell Carcinoma in a Patient with Membranous Glomerulonephritis / 영남의대학술지

Membranous glomerulonephritis can manifest as a paraneoplastic syndrome. The presence of evidence that supports the relationship between malignancy and membranous glomerulonephritis remains unresolved, though. Membranous glomerulonephritis has been commonly reported as associated with solid or hematologic malignancy, such as lung cancer, prostate cancer, and gastro-intestinal cancer, but its concomitant existence with skin cancer is rare. This paper reports a case of membranous glomerulonephritis combined with basal cell carcinoma that was successfully treated with the excision of the basal skin cell carcinoma.

Glomerulonefritis Membranosa asociada al uso de analgésicos antiinflamatorios no esteroideos: Reporte de caso / Membranous Glomerulonephritis associated with the use of non-steroidal anti-inflamatory drugs: Case report

Las alteraciones renales producidas por el uso crónico de analgésicos antiinflamatorios no esteroideos (AINE) son diversas, la principal corresponde a nefropatía tubulointersticial que resulta en la necrosis papilar renal. También se han reportado casos de síndrome nefrótico con biopsias que revelan enfermedad de cambios mínimos y muy excepcionalmente una glomerulonefritis membranosa, la cual desaparece al suspender el medicamento. Se presenta el caso de una paciente remitida al Servicio de Nefrología del Hospital Militar Central (HMC) de Bogotá por presentar elevación de los azoados durante una valoración prequirúrgica. Sin evidencia de causa secundaria y después de ser estudiada fue llevada a biopsia renal, encontrando una nefropatía membranosa y como único antecedente el uso crónico de AINE.

The renal effects caused by chronic use of non-steroidal anti-inflammatory drugs (NSAIDs) are diverse; the main corresponds to tubulointerstitial nephropathy resulting in renal papillary necrosis. There have also been reported cases of nephrotic syndrome with biopsy that revealed minimal change disease and exceptionally membranous glomerulonephritis, which resolves after discontinuation of medication. There is a report of a case in Bogota in which a patient was referred to the Nephrology Service of the Central Military Hospital due to elevation of nitrogen compounds during preoperative assessment. No evidence of secondary cause was present and after being studied, a renal biopsy was carried out, membranous nephropathy located; the only positive finding in the past medical history is the chronic use of NSAIDs.

A Case of Membranous Glomerulonephritis Presenting with Right Common Iliac Artery Thrombosis as the Initial Sign / 대한내과학회지

Most patients with nephrotic syndrome visit the hospital because of edema due to hypoalbuminemia induced by severe proteinuria. However, rare cases have reported arterial thrombosis as the main problem complicating nephrotic syndrome. Arterial thrombosis combined with nephrotic syndrome is rarer than venous thrombosis, and it usually develops during treatment with steroids or diuretics. Arterial thrombosis is rarely diagnosed as the initial sign of nephrotic syndrome. We report the case of a 38-year-old-woman with membranous glomerulonephritis presenting with right common iliac artery thrombosis as the initial sign.

A Case of Systemic Thrombolysis in the Membranous Glomerulonephritis Presenting as Acute Inferior Vena Cava and Renal Vein Thrombosis / 대한신장학회지

Thromboembolism is one of the common complications in nephrotic syndrome. The incidence of renal vein thrombosis in the setting of nephrotic syndrome varies from 5% to 62%. Membranous glomerulonephritis (MGN) has been rarely manifested as inferior vena cava (IVC) and/or renal vein thrombosis. We report a case of acute IVC and left renal vein thrombosis as initial manifestation of MGN. The renal vein thrombosis was successfully treated with systemic thrombolysis with temporary IVC filter insertion. After 4 months, MGN was diagnosed by renal biopsy.

Membranous glomerulonephritis associated with pancreatic tuberculosis / 대한내과학회지

A 51-year old man presented with generalized edema for 10 days. He was admitted to the hospital for a renal biopsy, and a pancreatic mass was incidentally detected by ultrasonography. The renal biopsy was consistent with membranous glomerulonephritis and the pancreas biopsy revealed chronic granulomatous inflammation with multinucleated giant cells, which was suggestive of tuberculosis of the pancreas. The patient was initially treated with a steroid and anti-tuberculosis drugs, but the proteinuria in the nephrotic range persisted throughout the 3-month follow-up. With the presumed diagnosis of tuberculosis-induced membranous nephropathy, immunosuppressant therapy was stopped and anti-tuberculosis drugs were administered for 31 months. With anti-tuberculous treatment, the proteinuria was reduced from 22 g/day to 0.57 g/day. A follow-up abdominal CT revealed a marked reduction in the size of the pancreatic mass. This finding suggests that the membranous nephropathy in our case was related to the pancreatic tuberculosis rather than it being related to primary nephrotic syndrome.

A Case of Membranous Glomerulonephritis in a Patient with HIV Infection / 대한신장학회지

Viral infections can be causative in many glomerular disease, and human immunodeficiency virus (HIV) infection is closely related to a collapsing focal segmental glomerulosclerosis (FSGS). This is known as HIV associated nephropathy (HIVAN) and is characterized clinically by proteinuria, often of sudden onset, with rapidly progressive renal dysfunction resulting in end stage renal disease (ESRD) over several months. Increasingly, other primary renal diseases are being described in HIV infected patients, including IgA nephropathy, an immune complex lupus-like neprhopathy, and tubulonephritis. We observed rare HIVAN case presenting membranous glomerulonephritis with nephrotic syndrome in a woman who was positive for HIV without hepatitis B viral infection. She was treated with Methylprednisolone 60 mg/day, zidovudine 600 mg/day, efavirenz 60 mg/day, and lamivudine 300 mg/day for 5 months. After treatment, proteinuria decreased from 4,092 mg/day to 419 mg/day and CD4 T cell count rose from 594/mL to 1,176/mL. The effectiveness and safety of corticosteroids in the treatment of HIVAN remained controversial but this case showed good response for steroid with triple antiviral therapy about HIVAN especially membranous glomerulonephritis.

Clinical Characteristics of Membranous Glomerulonephritis Developed after Renal Transplantation / 대한이식학회지

PURPOSE: Survival rate after renal transplantation has increased due to the development of new immunosuppressive agents and operative techniques. Therefore, chronic complications have increased. Membranous glomerulonephritis (MGN) is one of the common glomerular diseases diagnosed in transplanted kidneys. The exact impact of posttransplantation MGN on the risks for graft loss and long-term graft outcomes is not defined clearly. Risk factors to predict a poor outcome are not well established. METHODS: The retrospective analysis was performed in 20 patients with posttransplantation MGN based on renal biopsy among 2,375 patients who underwent kidney transplantation in Shinchon Severance Hospital from April 1979 to December 2003. RESULTS: After renal transplantation, five patients had de novo MGN and three patients had recurrent MGN. MGN was diagnosed by biopsy at 38.0+/-25.0 months after transplantation. (5~99 months) The duration of graft survival was 115.5+/-52.4 months. The lower was BUN level at 1 month after transplantation, the longer was the interval between renal transplantation and diagnosis of MGN. But donor age, dialysis duration, creatinine at diagnosis of MGN and immunosuppressive agents were not significantly related with the time from transplantation to diagnosis. In 10 cases, renal function was aggravated gradually and the other 10 cases, renal function remained stable. Graft loss occurred in 7 of 20 patients. Five of seven patients with graft loss transferred to peritoneal dialysis and the other 2 patients transferred to hemodialysis. CONCLUSION: De novo MGN and recurrent MGN can develop at any time after transplantation. Graft outcome is variable, with some patients progressing to graft failure, and others maintaining stable graft function. In conclusion, MGN after renal transplantation was more likely to occur in male. Aggressive evaluation such as renal biopsy will be needed when accompanied by hypertension and proteinuria. Renal function of de novo MGN will be maintained with proper immunosuppressive agents and conservative management.

A Case of Idiopathic Membranous Glomerulonephritis in association with Thin Glomerular Basement Membrane Nephropathy / 대한신장학회잡지

We report a rare case of the idiopathic membranous glomerulonephritis (IMGN) in association with the thin glomerular basement membrane nephropathy (TGBMN) in a 63-year-old female with hematuria. This is the first case reported in Korea. In renal biopsy of this case, direct immunofluorescence demonstrated anti-IgG Ab along the glomerular capillary wall with granular pattern. The basement membrane was thin, about 170-220 nm and small epimembranous electron dense deposits were observed by electron microscopy. As this case, the combination of TGBMN and IMGN is very uncommon because the IMGN is characterized morphologically by diffuse global thickening of the glomerular capillary wall, while the TGBMN is defined as an extreme thinning of the glomerular basement membrane, less than 200 nm. Our case showed no renal function deterioration and benign prognosis as other reports showed.

A Case of Guillain-Barre Syndrome associated with Membranous Glomerulonephritis and Uveitis

No abstract available.

Combined IgA Nephropathy and Membranous Glomerulonephritis : A Report of Six Cases

IgA nephropathy (IgAN) and membranous glomerulonephritis (MGN) are common in adults. However, it is unlikely that these two distinct glomerulonephrites coexist in a renal biopsy. Here, we report clinical and pathological data of six patients with concomitant existence of IgAN and MGN in renal biopsy specimens from 1990 to 2004. Five patients were male and one was female, and their ages ranged from 29 to 71 years. Four patients had microscopic hematuria, five had nephrotic range proteinuria, three had hepatitis B virus infections, three had rheumatoid factors, one had antinuclear antibodies. Two cases were developed after kidney transplant. Immunofluorescence microscopy showed characteristic findings of mesangial IgA deposits and granular IgG deposits on the capillary walls. These were confirmed by electron microscopic findings of immune-type electron-dense deposits in the mesangium and subepithelial capillary basement membranes. The pathogenesis and prognosis of the patients are discussed in this report.