When the finding of glomerular fibrils in patients with nephrotic syndrome leads to an erroneous diagnosis

Huerta, Ana; Segovia, Beatriz; Hernández, Ana Cristina Gallego; Morales, Enrique; Gonzalez, Jorge; Mérida, Eva; Martı́nez, Miguel Ángel; Praga, Manuel

doi:10.1093/ndtplus/sfn167

Cited by 2 publications

(1 citation statement)

References 15 publications

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“…It is beyond doubt, that the diagnosis of FGN may be missed if EM is not performed. In contrast, the presence of atypical segmental fibrillar deposits could be misleading, since fibrils are encountered in a series of other glomerular disorders, namely diabetic nephropathy, cryoglobulinemic glomerulonephritis and lupus nephritis [10] . The use of mass spectrometry and the discovery of the immunohistochemical biomarker DNAJB9 comprise promising tools for a more accurate diagnosis of FGN [20] .…”

Section: Discussionmentioning

confidence: 99%

Clinicopathologic features and treatment outcomes of patients with fibrillary glomerulonephritis

et al. 2021

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Fibrillary glomerulonephritis (FGN) is a diverse glomerular disease with poor renal prognosis. The optimal therapeutic approach remains undetermined, as treatment outcomes vary across different studies. We retrospectively reviewed the medical data of 10 patients diagnosed with biopsy-proven FGN at our center between 2004 and 2019. Clinical and histological features, as well as therapeutic regimens and treatment response, are reported. The patients were predominantly men (2.5/1 men-female ratio) with a mean age at diagnosis of 46.5 years (IQR: 41.5-59.5). The median proteinuria and creatinine levels at presentation were 2.55 g/day (IQR: 0.4-8.9) and 1.35 mg/dl (IQR: 0.94–1.88), respectively. Four out of 10 patients presented with nephrotic syndrome, 5 patients with nephritic syndrome and 1 with isolated microscopic hematuria. Light microscopy showed mesangial proliferative (n = 7), membranoproliferative-like (n = 2), and diffuse sclerosing patterns (n = 1). Rituximab was used in 7/10 patients, either as monotherapy (n = 3) or combined with cyclophosphamide and corticosteroids (n = 4). Patients who were treated with immunosuppression had higher median levels of creatinine (1.40 mg/dl) and proteinuria (3.5 g/d) compared to those who received supportive treatment alone (0.94 mg/dl and 0.6 g/d, respectively). After a median follow-up of 30 months (IQR:18–66.5), 4 out of 7 patients (57%) treated with immunosuppression achieved a clinical response, 1 had persistent renal dysfunction and 2 patients progressed to end-stage renal disease. The present case series extends the existing literature on the clinical features and outcomes of FGN, as well as the use of rituximab-based regimens for the treatment of the disease. Further research is needed to establish the proper management of the disease.

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Section: Discussionmentioning

confidence: 99%

Clinicopathologic features and treatment outcomes of patients with fibrillary glomerulonephritis

et al. 2021

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Effect size of Dna-j heat shock protein family B member 9 (DNAJB9) biomarker in kidney biopsy specimens on kidney outcomes in fibrillary glomerulonephritis

Amiri

2022

KIDNEYS

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Background. Fibrillary glomerulonephritis is a rare glomerular disease that presents with hypertension, hematuria, nephrotic syndrome and renal insufficiency. The purpose of this research was to assess effect of DNAjB9 staining marker in kidney biopsy specimens on kidney outcomes. Materials and methods. In this analytic (experimental) clinical study with randomized clinical trial design in meta-analysis article, 72 patients with biopsy-proven fibrillary glomerulonephritis were investigated. Clinical features, laboratory data at initial presentation, management and outcomes were collected. The paper has written based on searching PubMed Central and Google Scholar to identify potentially relevant articles. Median, percentage, mean ± standard deviation (SD), two-tailed t and Chi-square, two proportion difference meta-analysis and Kaplan-Meier analysis were used for statistical evaluation. Moreover, relative risk, odds ratio, Spearman’s correlation for statistical analyses were used. Results. The median and interquartile range of age of patients with fibrillary nephropathy at the time of diagnosis were 55 and 18 years, respectively. There was no statistically significant difference between two sex groups of males and females in current research (p-value: 0.35). There was significant statistical correlation between elevated serum creatinine level and time of last serum creatinine measurement with p-value of 0.01 and confidence interval (CI) of 0.7820 to –0.1258 during follow-up. Relative risk of kidney failure progression to kidney replacement therapy (↑ ≥ 2-fold in serum creatinine or dialysis or kidney transplant) in DNAjB9-positive (group I) and DNAjB9-negative patients (group II) was assessed 2.67 with 95% CI of 1.128 to 6.3044 and p-value of 0.025. Odds ratio of kidney failure progression to kidney replacement therapy (↑ ≥ 2-fold in serum creatinine or dialysis or kidney transplant) was assessed 4.33 with 95% CI of 0.9464 to 19.8417 and p-value of 0.058. There was statistically significant difference when comparing group I and group II for mortality probability (Kaplan-Meier analysis) during follow-up (P < 0.0001). Conclusions. The present study revealed high mortality in DNAjB9-negative (8/64, 12.5%) versus DNAjB9-positive patients (0/8) with statistically significant level. Relative risk and odds ratio of kidney failure progression to kidney replacement therapy were assessed 2.67 and 4.33, respectively.

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When the finding of glomerular fibrils in patients with nephrotic syndrome leads to an erroneous diagnosis

Cited by 2 publications

References 15 publications

Clinicopathologic features and treatment outcomes of patients with fibrillary glomerulonephritis

Clinicopathologic features and treatment outcomes of patients with fibrillary glomerulonephritis

Effect size of Dna-j heat shock protein family B member 9 (DNAJB9) biomarker in kidney biopsy specimens on kidney outcomes in fibrillary glomerulonephritis

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