Edit concept Question Editor Create issue ticket

Idiopathic Membranous Glomerulonephritis


Presentation

  • The median age at presentation was 36. Fifty-four percent of patients had the nephrotic syndrome at presentation. Half of the patients were treated at some stage with cyclophosphamide or corticosteroids.[ncbi.nlm.nih.gov]
  • Those with 'progressive' MGN had heavier proteinuria and worse renal function on presentation, but the overlap was considerable. Patients with progressive MGN were treated after 1-4 years.[ncbi.nlm.nih.gov]
  • Poor prognostic indicators were: nephrotic syndrome at presentation, impaired function at time of diagnosis, male patients and older age.[ncbi.nlm.nih.gov]
  • 2 brothers with identical HLA antigens presented an idiopathic membranous glomerulonephritis. This is, to our knowledge, the first report of familial membranous glomerulonephritis.[ncbi.nlm.nih.gov]
  • Abstract Here we present a rare report of diffuse thin glomerular basement membrane (dTGBM) with idiopathic membranous glomerulonephritis (IMGN). dTGBM was found in 11 (2.04%) of 539 adult patients with IMGN. The male:female ratio was 1:1.2.[ncbi.nlm.nih.gov]
Weakness
  • The correlations between interstitial expression of alpha-SMA and interstitial volume, CD45RB , CD43 , CD20 as well as CD68 , cells were in MCD patients weak and not significant.[ncbi.nlm.nih.gov]
  • Correlations between proteinuria and total glomerular cells per unit of glomerular area, were weak and not significant in both MCD and MGN groups.[revistanefrologia.com]
  • The initial sample shows weak reactivity for native HGE and recombinant PLA2R. Insignificant amount of reactivity was detected in his follow-up sample (lower).[karger.com]
  • The evidence that such therapy is beneficial in MN is weak and largely inferential, and the following issues need to be considered when using an ACEi and or an ARB in patients with MN: ( 1 ) In both diabetic and nondiabetic nephropathy patients, the use[cjasn.asnjournals.org]
Pathologist
  • Assessment of these factors thus allows the pathologist to make a relevant statement concerning the probable course and prognosis of the disease at the time of the diagnostic biopsy.[ncbi.nlm.nih.gov]
  • This detection of enlarged glomeruli may serve to alert the renal pathologist to the possibility of coexisting FSGS, which is a poor prognostic factor in IMGN.[ncbi.nlm.nih.gov]
Suggestibility
  • The fact that both brothers had identical HLA antigens suggests a genetic predisposing background.[ncbi.nlm.nih.gov]
  • As progressive deterioration in renal function in membranous glomerulonephritis is associated with persistent heavy proteinuria these results suggest a beneficial effect of treatment.[ncbi.nlm.nih.gov]
  • The results suggest that complement activation through the classical pathway usually occurs in IMGN. The detection of C4d and C4bp in the glomeruli appears to be a sensitive indicator of the classical pathway activation in IMGN.[ncbi.nlm.nih.gov]
  • This case suggests that long-term LDL-A therapy may be a treatment option for idiopathic MGN refractory to immunosuppressive therapy or short-term LDL-A.[ncbi.nlm.nih.gov]
  • It has been suggested that due to a long-term immunomodulator therapy the level of urinary protein excretion might be reduced in 77% of patients.[ncbi.nlm.nih.gov]
Microscopic Hematuria
  • Symptoms and Signs Patients typically present with edema and nephrotic-range proteinuria and occasionally with microscopic hematuria and hypertension. Symptoms and signs of a disorder causing MN (eg, a cancer) may be present initially.[merckmanuals.com]
  • Moreover, adults show an increased incidence of atypical clinical features such as hypertension, renal function impairment, non-selective proteinuria and microscopic hematuria 2, 3.[revistanefrologia.com]
  • Mild microscopic hematuria may be present in MN (both primary and secondary). A 24 hour urine collection should be obtained to quantify the amount of proteinuria.[renalandurologynews.com]
  • Severe hypoproteinemia (albumin 3.5 g/l) in 30 (41.1%), microscopic hematuria in 15 (20.5%), accompanying hypertension in 23 (31.5%), and eGFR 2 at the time of renal biopsy in 26 (35.6%) patients.[spandidos-publications.com]
  • The presence of microscopic hematuria is common (30 to 40%), but macroscopic hematuria and red cell casts are rare and suggest a different diagnosis ( 13 ).[cjasn.asnjournals.org]
Neglect
  • For this study biopsies containing less than 10 nonsclerotic glomeruli were neglected. Immunofluorescence microscopy Tissue was snap frozen, sectioned at 5 µ and fixed in 95% alcohol for 10 min.[revistanefrologia.com]

Workup

  • Unlike these cases, our patient's workup did not reveal any underlying etiology for secondary MN.[karger.com]
  • Secondary causes of MN were excluded after clinical workup including detailed medical history, clinical examination, analysis like lupus antibodies, hepatitis B, C and HIV, and at least chest X-ray and abdominal ultrasound.[scielo.mec.pt]
  • The two can be distinguished by clinical, laboratory, and histological features (see Presentation and Workup ). [1] Successful treatment of the underlying cause may be curative in secondary forms.[emedicine.medscape.com]
  • An infectious workup, including blood and urine cultures, was negative and the symptoms resolved with only supportive care. A specific virus was not isolated, but the course was most consistent with a viral process.[bmcnephrol.biomedcentral.com]
  • The patient underwent extensive serologic workup including monoclonal protein studies, serum complement levels, anti-neutrophil cytoplasmic antibodies, hepatitis B and C and HIV serology, and anti-nuclear antibody, all of which were negative or in the[cjasn.asnjournals.org]
Creatinine Increased
  • The patients (six male, four female, mean age 50 years) were monitored over 9-30 months during which time creatinine clearance reduced from (mean /- SEM) 83 /- 10 to 29 /- 6 ml/min, and plasma creatinine increased from 135 /- 22 to 297 /- 35 mumol/l.[ncbi.nlm.nih.gov]

Treatment

  • A significantly greater improvement in urinary protein excretion was, however, observed at all time points in the treatment group. Plasma albumin was also significantly higher in the treatment group at 18 and 24 months.[ncbi.nlm.nih.gov]
  • Rituximab is effective in treatment of idiopathic retroperitonea lfibrosis with positivity of CD20 cells, as well as in treatment of idiopathic membranous glomerulonephritis.[ncbi.nlm.nih.gov]
  • First, in each case a long-term prednisone treatment was administered. During this therapy 12 (40%) patients improved and 9 had remission. In the 18 (60%) steroid resistant cases the treatment was combined with cytostatic drugs.[ncbi.nlm.nih.gov]
  • Treatment administered was pulse intravenous methyl-prednisolone 1 g X 3 then oral prednisolone 30 mg and azathioprine 50 mg (nine patients) or cyclophosphamide 50 mg (one patient).[ncbi.nlm.nih.gov]
  • In this study azathiprine appeared not to be useful in the treatment of idiopathic membranous glomerulonephritis.[ncbi.nlm.nih.gov]

Prognosis

  • Assessment of these factors thus allows the pathologist to make a relevant statement concerning the probable course and prognosis of the disease at the time of the diagnostic biopsy.[ncbi.nlm.nih.gov]
  • It was concluded that Japanese cases had an obviously better prognosis despite having more advanced histological findings than the caucasian patients.[ncbi.nlm.nih.gov]
  • Abstract A retrospective long-term study (average follow-up time 5.2 years) of 334 patients with idiopathic membranous glomerulonephritis (MGN) was carried out with the following results: 1) MGN was found to have a relatively good prognosis when all cases[ncbi.nlm.nih.gov]
  • These data are in accord with other recently published series which have described a relatively benign prognosis for idiopathic membranous glomerulonephritis.[ncbi.nlm.nih.gov]
  • These findings suggest, that in MGN mainly interstitial lesions are involved in the renal prognosis and they are not related to the severity of glomerular changes.[ncbi.nlm.nih.gov]

Etiology

  • A careful medication history and renal biopsy were essential in identifying the etiology of the acute kidney injury in this patient with a complex renal history.[ncbi.nlm.nih.gov]
  • Hence a reserved view must be taken when treatment with potentially dangerous agents is considered in a disease with unknown etiology and pathogenesis.[ncbi.nlm.nih.gov]
  • Unlike these cases, our patient's workup did not reveal any underlying etiology for secondary MN.[karger.com]
  • Ongoing controversies regarding etiology, diagnosis, treatment 1. The role of M-type phospholipase A2 receptor and antibodies in pediatric patients with IMN is unknown. 2.[clinicaladvisor.com]
  • Lancet (1975) [ Pubmed ] Effect of protein A immunoadsorption in nephrotic syndrome of various etiologies. Esnault, V.L., Besnier, D., Testa, A., Coville, P., Simon, P., Subra, J.F., Audrain, M.A. J. Am. Soc.[wikigenes.org]

Epidemiology

  • Epidemiology and clinical outcomes of patients with idiopathic membranous glomerulonephritis at Groote Schuur Hospital over a ten year period. University of Cape Town. Includes bibliographical references[open.uct.ac.za]
  • Membranous nephropathy in the older adult: epidemiology, diagnosis and management. Drugs Aging. 2007; 24(9): 717-32.[arupconsult.com]
  • View Article : Google Scholar : PubMed/NCBI 4 Li LS and Liu ZH: Epidemiologic data of renal diseases from a single unit in China: Analysis based on 13,519 renal biopsies. Kidney Int. 66:920–923. 2004.[spandidos-publications.com]
  • In an epidemiologic biopsy study of patients with HIV and renal disease, Szczech et al. [ 1 ] described HIV-positive patients with MN benefitting primarily from treatments other than HAART.[karger.com]
  • Detailed epidemiology: The incidence of MN is low in children and it is documented in 1-5% of all kidney biopsies performed in pediatric patients with proteinuria or nephrotic syndrome.[clinicaladvisor.com]
Sex distribution
Age distribution

Pathophysiology

  • From basic science and pathophysiology to clinical best practices, Brenner & Rector’s The Kidney is your go-to resource for any stage of your career.[books.google.de]
  • Brenner/Rector remains the go-to resource for practicing and training nephrologists and internists who wish to master basic science, pathophysiology, and clinical best practices.[books.google.de]
  • "The finding not only furthers our understanding of the pathophysiological basis of membranous nephropathy, but also allows for the potential identification and monitoring of patients who are positive for anti-THSD7A autoantibodies, by both serologic[medpagetoday.com]
  • [Membranous nephropathy: Diagnosis, new insights in pathophysiology, and therapeutic approach]. Rev Med Interne. 2016 May 25. [Medline]. Tran TH, J Hughes G, Greenfeld C, Pham JT.[emedicine.medscape.com]
  • Definition / general Most common cause of nephrotic syndrome in adults (40%); 5% of cases in children Diffuse glomerular wall thickening due to in situ immune complexes (electron dense) in glomerular basement membrane but NOT in mesangium Pathophysiology[pathologyoutlines.com]

Prevention

  • Although there have been considerable advances in the management of renal failure by dialysis and transplantation, there has been relatively little progress in its prevention.[books.google.de]
  • […] tacrolimus and mycophenolate mofetil tacrolimus 2mg bd adjusted to achieve levels of 5-12ng/L mycophenolate mofetil 500mgs bd adjusted to achieve levels of 1.5-3mg/L Other Names: prograf cellcept Primary Outcome Measures : Efficacy of mycophenolate in preventing[clinicaltrials.gov]
  • Research is still trying to establish the best treatments to control membranous nephropathy and prevent it from causing kidney failure.[edren.org]
  • Is chronic lung disease in low birth weight infants preventable? A survey of eight centers. ‎ Página 84 - Tanner JM, Whitehouse RH, Marubini E, Resele LF. The adolescent growth spurt of boys and girls of the Harpenden growth study. ‎[books.google.es]
  • How can membranous glomerulopathy be prevented? The cause of IMN is uncertain is most cases and cannot be prevented by prophylactic drugs or vaccinations. Secondary cases of MN are the consequence of the underlying disease.[clinicaladvisor.com]

Ask Question

5000 Characters left Format the text using: # Heading, **bold**, _italic_. HTML code is not allowed.
By publishing this question you agree to the TOS and Privacy policy.
• Use a precise title for your question.
• Ask a specific question and provide age, sex, symptoms, type and duration of treatment.
• Respect your own and other people's privacy, never post full names or contact information.
• Inappropriate questions will be deleted.
• In urgent cases contact a physician, visit a hospital or call an emergency service!