Fibrillary glomerulonephritis with prevalent IgA deposition associated with undifferentiated connective tissue disease: A case report

Nebuloni, Manuela; Genderini, A.; Tosoni, Antonella; Caruso, Sabrina; Belgiojoso, G. Barbiano di

doi:10.1093/ndtplus/sfp125

Cited by 3 publications

(2 citation statements)

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“…The availability of DNAJB9 immunohistochemical stain, a highly sensitive and specific marker for FGN [ 4–6 ], now allows for easier diagnosis of FGN. Of note, there have been two reported cases of IgA-dominant FGN [ 24 , 25 ], which are different than our cases in that they did not exhibit IgG staining on IF or granular electron-dense deposits on EM. Thus, these two cases do not represent FGN–IgAN but rather exceptional cases of an IgA variant of FGN.…”

Section: Discussioncontrasting

confidence: 91%

Characteristics of patients with coexisting DNAJB9-associated fibrillary glomerulonephritis and IgA nephropathy

Said

Rocha

Valeri

et al. 2020

Clinical Kidney Journal

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Background Coexistence of fibrillary glomerulonephritis (FGN) and immunoglobulin A (IgA) nephropathy (IgAN) in the same kidney biopsy (FGN–IgAN) is rare, and the clinicopathologic characteristics and outcome of this dual glomerulopathy are unknown. Methods In this study, 20 patients with FGN–IgAN were studied and their characteristics were compared with 40 FGN and 40 IgAN control patients. Results Concurrent IgAN was present in 1.8% of 847 consecutive FGN cases and was the second most common concurrent glomerulopathy after diabetic nephropathy. FGN–IgAN patients were overwhelmingly White (94%) and contrary to FGN patients were predominantly (60%) males. Compared with IgAN patients, FGN–IgAN patients were older, had higher proteinuria, a higher incidence of renal insufficiency, and a lower incidence of microhematuria and gross hematuria at diagnosis. Six (30%) patients had malignancy, autoimmune disease or hepatitis C infection, but none had a secondary cause of IgAN or clinical features of Henoch–Schonlein purpura. Histologically, all cases exhibited smudgy glomerular staining for immunoglobulin G and DnaJ homolog subfamily B member 9 (DNAJB9) with corresponding fibrillary deposits and granular mesangial staining for IgA with corresponding mesangial granular electron-dense deposits. On follow-up (median 27 months), 10 of 18 (56%) FGN–IgAN patients progressed to end-stage kidney disease (ESKD), including 5 who subsequently died. Serum creatinine at diagnosis was a poor predictor of renal survival. The proportion of patients reaching ESKD or died was higher in FGN–IgAN than in IgAN. The median Kaplan–Meier ESKD-free survival time was 44 months for FGN–IgAN, which was shorter than IgAN (unable to compute, P = 0.013) and FGN (107 months, P = 0.048). Conclusions FGN–IgAN is very rare, with clinical presentation and demographics closer to FGN than IgAN. Prognosis is guarded with a median renal survival of 3.6 years. The diagnosis of this dual glomerulopathy requires careful evaluation of immunofluorescence findings, and electron microscopy or DNAJB9 immunohistochemistry.

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Section: Discussioncontrasting

confidence: 91%

Characteristics of patients with coexisting DNAJB9-associated fibrillary glomerulonephritis and IgA nephropathy

Said

Rocha

Valeri

et al. 2020

Clinical Kidney Journal

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“…[2] The association between IgA deposition and FGN/ITG, as reported in the present case, has been previously described. [21][22][23][24][25] The results of the bone marrow puncture in our case did not suggest hematological disease. Some studies have reported that monoclonal FGN is not associated with abnormal proteinemia or hematolymphoid neoplasms.…”

Section: Discussionmentioning

confidence: 94%

Diagnosis of Fibrillary Glomerulonephritis with Positive Immunoglobulin A-κ Deposits Presenting as Membranous Nephropathy Using Immunoelectron Microscopy: A Case Report and Literature Review

Li¹,

Yan²,

Liu³

2023

Integr Med Nephrol Androl

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Fibrillary glomerulonephritis (FGN) is a rare disease characterized by randomly arranged fibrils with a diameter of 10–30 nm on immunoelectron microscopy. Clinically, renal insufficiency, hypertension, hematuria, and proteinuria are the typical symptoms of FGN. Currently, there are limited treatment options for FGN, and transplant recurrence is common. We reported the case of a middle-aged Chinese woman diagnosed with FGN with positive immunoglobulin A-κ deposits presenting as membranous nephropathy.

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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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Fibrillary glomerulonephritis with prevalent IgA deposition associated with undifferentiated connective tissue disease: A case report

Cited by 3 publications

References 4 publications

Characteristics of patients with coexisting DNAJB9-associated fibrillary glomerulonephritis and IgA nephropathy

Characteristics of patients with coexisting DNAJB9-associated fibrillary glomerulonephritis and IgA nephropathy

Diagnosis of Fibrillary Glomerulonephritis with Positive Immunoglobulin A-κ Deposits Presenting as Membranous Nephropathy Using Immunoelectron Microscopy: A Case Report and Literature Review

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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