Crescentic fibrillary glomerulonephritis associated with hepatitis C viral infection

Guerra, Grazia Maria; Narayan, Girish; Rennke, H. G.; Jaber, Bertrand L.

doi:10.5414/cnp60364

Cited by 31 publications

(19 citation statements)

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“…The pathogenetic link between FGN and carcinoma is less clear and remains to be proven; thus far, no tumor antigens have been shown in the glomerular fibrillar deposits. Only two of our patients (3%) had chronic hepatitis C infection, which has been previously reported in few patients (7,21,22). Positive M-spike on SPEP and/or UPEP was present in 17% of our patients, including six who fulfilled the diagnostic criteria for MM, and in 15% of patients in the series by Rosenstock et al (2), justifying the need to exclude an underlying dysproteinemia in all patients who are diagnosed with FGN.…”

Section: Discussionsupporting

confidence: 59%

“…The light microscopic features are heterogenous; most cases exhibit mesangial expansion/hypercellularity with or without duplication of the GBMs (2,3). Less commonly reported morphologic patterns included endocapillary proliferative glomerulonephritis (EPGN) and crescentic glomerulonephritis (CGN) (2,7). By definition, the glomerular deposits in FGN are Congo red-negative, which distinguishes it from amyloid.…”

Section: Introductionmentioning

confidence: 99%

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Fibrillary Glomerulonephritis

Nasr

Valeri

Cornell

et al. 2011

Clinical Journal of the American Society of Nephrology

187

293

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SummaryBackground and objectives Fibrillary glomerulonephritis (FGN) is a rare primary glomerular disease. Most previously reported cases were idiopathic. To better define the clinical-pathologic spectrum and prognosis, we report the largest single-center series with the longest follow-up.Design, setting, participants, & measurements The characteristics of 66 FGN patients who were seen at Mayo Clinic, Rochester, between 1993 and 2010 are provided. ResultsThe mean age at diagnosis was 53 years. Ninety-five percent of patients were white, and the female: male ratio was 1.2:1. Underlying malignancy (most commonly carcinoma), dysproteinemia, or autoimmune disease (most commonly Crohn's disease, SLE, Graves' disease, and idiopathic thrombocytopenic purpura), were present in 23, 17, and 15% of patients, respectively. Presentation included proteinuria (100%), nephrotic syndrome (38%), renal insufficiency (66%), hematuria (52%), and hypertension (71%). The most common histologic pattern was mesangial proliferative/sclerosing GN followed by membranoproliferative GN. During an average of 52.3 months of follow-up for 61 patients with available data, 13% had complete or partial remission, 43% had persistent renal dysfunction, and 44% progressed to ESRD. The disease recurred in 36% of 14 patients who received a kidney transplant. Independent predictors of ESRD by multivariate analysis were older age, higher creatinine and proteinuria at biopsy, and higher percentage of global glomerulosclerosis.Conclusions Underlying malignancy, dysproteinemia, or autoimmune diseases are not uncommon in patients with FGN. Prognosis is poor, although remission may occur in a minority of patients without immunosuppressive therapy. Age, degree of renal impairment at diagnosis, and degree of glomerular scarring are predictors of renal survival.

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Section: Discussionsupporting

confidence: 59%

Section: Introductionmentioning

confidence: 99%

Fibrillary Glomerulonephritis

Nasr

Valeri

Cornell

et al. 2011

Clinical Journal of the American Society of Nephrology

187

293

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“…Fibrillary Glomerulonephritis has been found to be idiopathic in some studies but other studies have found it to be associated with malignancy (mainly carcinoma), autoimmune diseases (mainly Crohn's disease, systemic lupus erythematosus, Graves' disease, and idiopathic thrombocytopenic purpura) and hepatitis-C virus infection (2,4,6,(19)(20)(21)(22)(23)(24)(25). The clinical presentation of FG in childhood usually involves asymptomatic proteinuria and/or microscopic hematuria but pediatric clinical presentations of MPGN, acute poststreptococcal glomerulonephritis and systemic lupus erythematosus at the beginning of the disease have also been reported (1,(7)(8)(9)(10)(11)20).…”

Section: Discussionmentioning

confidence: 99%

Unusual Fibrillary Glomerulonephritis in a 19-Month-Old Male Patient: A Case Report and Review of the Literature

Zeybek¹,

Bolat²,

Orman³

et al. 2017

Turk Neph Dial Transpl

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Fibrillary glomerulonephritis (FG) is an immune-mediated type of glomerulonephritis characterized by Congo red-negative fibrillary electron-dense deposits. The incidence of FG is less than 1% in adults and it is very rare in children. We herein present a case of FG in a child with steroid-resistant nephrotic syndrome (SRNS). Nephrotic syndrome was diagnosed in a 19-month-old male patient based on the presence of periorbital edema and nephrotic-range proteinuria. Renal biopsy was performed because of the lack of response to eight weeks of prednisolone treatment and FG was diagnosed by electron microscopy. Unusual IgM deposits were present on immunofluorescence microscopy, contrary to previous reports in the literature. Because steroids and cytotoxic drugs were ineffective for the treatment of FG, an angiotensin-converting enzyme inhibitor (ACEi) was started. Monotherapy with the ACEi, caused the proteinuria to fall from the nephrotic-range to the subnephrotic-range. This case illustrates that although very rare in childhood, FG should be considered as a differential diagnosis of SRNS. Additionally, the prognosis of FG childhood is much better than that of FG in adulthood. ACEi therapy alone may be considered in the treatment of FG in childhood. ÖZFibriler glomerülonefrit (FG), Kongo-kırmızısı boyası negatif boyanan, fibriler elektron-yoğun birikimlerle karakterize immün-aracılı glomerülonefrit tipidir. FG insidansı erişkinlerde %1'in altındadır ve çocuklarda da çok nadirdir. Biz burada steroid-rezistan nefrotik sendrom (SRNS) ayırıcı tanısında FG saptadığımız bir çocuk olguyu sunduk. Ondokuz aylık erkek hastada periorbital ödem ve nefrotik düzeyde proteinüri varlığı nedeniyle nefrotik sendrom tanısı konuldu. Sekiz haftalık prednizolon tedavisiyle remisyon sağlanamayınca renal biyopsi uygulandı ve elektron mikroskopi bulgularıyla FG tanısı konuldu. İmmünfloresan mikroskopide, daha önce literatürde bildirilenin aksine yoğun IgM birikimi mevcuttu. Steroid ve sitotoksik ilaçlar FG tedavisinde etkisiz oldukları için hastaya anjiotensin-dönüştürücü enzim inhibitörü (ADEi) tedavisi başlandı. ADEi ile yapılan monoterapi, nefrotik düzeyde proteinürinin subnefrotik düzeylere düşmesine neden oldu. Bu olgu bize, her ne kadar çocukluk çağında çok nadir olsa da SRNS ayırıcı tanısında FG'i de düşünmemizi gösterir. Ayrıca, çocukluk çağı FG'inin prognozu erişkin çağı FG'inden daha iyidir. Tek başına ADEi tedavisi, çocukluk çağı FG tedavisinde düşünülebilir.AnAHtAR SÖZCükleR: Fibriler glomerülonefrit, Çocukluk çağı steroid-rezistan nefrotik sendrom, Anjiotensin-dönüştürücü enzim inhibitörleri

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“…The source of immune stimulation remains obscure. 17 There is, as yet, no definitive treatment for FGN, and the response to treatment is generally poor, with more than half of the patients developing end-stage renal disease within 4 years. Features associated with poor renal outcome include older age, higher creatinine level at biopsy, higher 24-h urinary protein level at biopsy, and higher percentage of globally sclerotic glomeruli.…”

Section: Discussionmentioning

confidence: 99%

“…6 There have also been rare case reports of FGN associated with systemic lupus erythematosus (SLE), rheumatoid arthritis, primary biliary cirrhosis, hemolytic anemia, HIV infection, essential thrombocytosis, Castleman's disease, gastric adenocarcinoma, and metastatic hepatocellular carcinoma. 4,[10][11][12][13][14][15][16][17] To our knowledge, this is the first report of FGN in a patient with Behçet's syndrome.…”

Section: Case Reportmentioning

confidence: 99%

Fibrillary Glomerulonephritis Associated with Behçet’s Syndrome

et al. 2012

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Fibrillary glomerulonephritis (FGN) is a rare cause of progressive renal dysfunction resulting in fibrillary deposits in the mesangium and/or glomerular basement membrane (GBM). Some case reports have shown FGN in patients with rheumatoid arthritis and other autoimmune diseases. This is the first case report of FGN in a patient with Behçet's syndrome. The most common renal histological finding in Behçet's syndrome is secondary amyloidosis. A 46-year-old woman with a 4-year history of Behçet's syndrome was referred to the nephrology clinic with foamy urine with nonselective proteinuria (urine protein-to-creatinine ratio was 1400 mg protein/g creatinine) and microscopic hematuria. Serum and urine protein electrophoresis showed no evidence of monoclonal gammopathy. A renal biopsy was performed. Light microscopy showed mesangial widening and nodular expansion with hyaline deposits. Immunofluorescence microscopy revealed immunoglobulin M deposits in the mesangium. Congo red staining was negative. Electron microscopy showed fibrillary deposits on the GBM. Pathological findings were consistent with FGN. She had been taking 50 mg azathioprine and 3000 mg mesalazine per day for 4 years due to Behçet's syndrome, so we did not add any other immunosuppressive agents or corticosteroids. Treatment of this glomerulopathy is not promising. It has been noted that none of the various approaches, including corticosteroid, plasmapheresis, and cytotoxic therapy, improves prognosis.

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Crescentic fibrillary glomerulonephritis associated with hepatitis C viral infection

Cited by 31 publications

References 0 publications

Fibrillary Glomerulonephritis

Fibrillary Glomerulonephritis

Unusual Fibrillary Glomerulonephritis in a 19-Month-Old Male Patient: A Case Report and Review of the Literature

Fibrillary Glomerulonephritis Associated with Behçet’s Syndrome

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