Anti-Neurofascin–Associated Nephrotic-Range Proteinuria in Chronic Inflammatory Demyelinating Polyneuropathy

Bukhari, Syed Muzaffar Hussain; Bettin, Margaret; Cathro, Helen P.; Gwathmey, Kelly; Gautam, Jitendra; Bowman, Brendan

doi:10.1016/j.xkme.2020.06.016

Cited by 13 publications

(8 citation statements)

References 18 publications

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“…Accordingly, patients with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) may develop membranous glomerulonephritis (12-20). Therefore, the kidney is also a target for IgG4 autoantibodies in these patients (21, 22). Histopathological characteristics of IgG4-RLD were investigated in a kidney biopsy of one CIDP patient with CNTN1/Caspr1-complex autoantibodies, nephrotic syndrome and microhaematuria.…”

Section: Resultsmentioning

confidence: 99%

“…Therefore, the kidney is also a target for IgG4 autoantibodies in these patients (21,22). Histopathological characteristics of IgG4-RLD were investigated in a kidney biopsy of one CIDP patient with CNTN1/Caspr1-complex autoantibodies, nephrotic syndrome and microhaematuria.…”

Section: Serum Igg4 Levels Did Not Correlate With Anti-neuronal/neuromuscular Autoantibody Titresmentioning

confidence: 99%

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Anti-neuronal IgG4 autoimmune diseases and IgG4-related diseases may not be part of the same spectrum: a comparative study

Endmayr

Tunc

Ergin

et al. 2021

Preprint

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Background: IgG4 is associated with two emerging groups of rare diseases: 1) IgG4 autoimmune diseases (IgG4-AID) and 2) IgG4-related diseases (IgG4-RLD). Anti-neuronal IgG4-AID include MuSK myasthenia gravis, LGI1- and Caspr2-encephalitis and autoimmune nodo-/paranodopathies (CNTN1 or NF155 antibodies). IgG4-RLD is a multiorgan disease hallmarked by tissue-destructive fibrotic lesions with lymphocyte and IgG4 plasma cell infiltrates and increased serum IgG4 concentrations. It is unclear, whether IgG4-AID and IgG4-RLD share relevant clinical and immunopathological features. Methods: We collected and analysed serological, clinical, and histopathological data in 50 patients with anti-neuronal IgG4-AID and 16 patients with IgG4-RLD. Results: A significantly higher proportion of IgG4-RLD patients had serum IgG4 elevation when compared to IgG4-AID patients (50% vs. 16%, p = .015). Moreover, those IgG4-AID patients with elevated IgG4 did not meet the diagnostic criteria of IgG4-RLD, and their autoantibody titres did not correlate with their serum IgG4 concentrations. In addition, patients with IgG4-RLD were negative for anti-neuronal/neuromuscular autoantibodies and among these patients, men showed a significantly higher propensity for IgG4 elevation, when compared to women (p = .041). Last, a kidney biopsy from a patient with autoimmune paranodopathy due to CNTN1/Caspr1-complex IgG4 autoantibodies and concomitant nephrotic syndrome did not show fibrosis or IgG4+ plasma cells, which are diagnostic hallmarks of IgG4-RLD. Conclusion: Our observations suggest that anti-neuronal IgG4-AID and IgG4-RLD are most likely distinct disease entities.

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Section: Resultsmentioning

confidence: 99%

Section: Serum Igg4 Levels Did Not Correlate With Anti-neuronal/neuromuscular Autoantibody Titresmentioning

confidence: 99%

Anti-neuronal IgG4 autoimmune diseases and IgG4-related diseases may not be part of the same spectrum: a comparative study

Endmayr

Tunc

Ergin

et al. 2021

Preprint

View full text Add to dashboard Cite

show abstract

“…Accordingly, patients with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) may develop membranous glomerulonephritis (17)(18)(19)(20)(21)(22)(23)(24)(25). Therefore, the kidney is also a target for IgG4 autoantibodies in these patients (26,27). Histopathological characteristics of IgG4-RLD were investigated in a kidney biopsy of one CIDP patient (male, 54 years) with CNTN1/Caspr1-complex autoantibodies, nephrotic syndrome and microhematuria.…”

Section: Lack Of Overlap Between Igg4-aid and Igg4-rldmentioning

confidence: 99%

Anti-Neuronal IgG4 Autoimmune Diseases and IgG4-Related Diseases May Not Be Part of the Same Spectrum: A Comparative Study

et al. 2022

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BackgroundIgG4 is associated with two emerging groups of rare diseases: 1) IgG4 autoimmune diseases (IgG4-AID) and 2) IgG4-related diseases (IgG4-RLD). Anti-neuronal IgG4-AID include MuSK myasthenia gravis, LGI1- and Caspr2-encephalitis and autoimmune nodo-/paranodopathies (CNTN1/Caspr1 or NF155 antibodies). IgG4-RLD is a multiorgan disease hallmarked by tissue-destructive fibrotic lesions with lymphocyte and IgG4 plasma cell infiltrates and increased serum IgG4 concentrations. It is unclear whether IgG4-AID and IgG4-RLD share relevant clinical and immunopathological features.MethodsWe collected and analyzed clinical, serological, and histopathological data in 50 patients with anti-neuronal IgG4-AID and 19 patients with IgG4-RLD.ResultsA significantly higher proportion of IgG4-RLD patients had serum IgG4 elevation when compared to IgG4-AID patients (52.63% vs. 16%, p = .004). Moreover, those IgG4-AID patients with elevated IgG4 did not meet the diagnostic criteria of IgG4-RLD, and their autoantibody titers did not correlate with their serum IgG4 concentrations. In addition, patients with IgG4-RLD were negative for anti-neuronal/neuromuscular autoantibodies and among these patients, men showed a significantly higher propensity for IgG4 elevation, when compared to women (p = .005). Last, a kidney biopsy from a patient with autoimmune paranodopathy due to CNTN1/Caspr1-complex IgG4 autoantibodies and concomitant nephrotic syndrome did not show fibrosis or IgG4+ plasma cells, which are diagnostic hallmarks of IgG4-RLD.ConclusionOur observations suggest that anti-neuronal IgG4-AID and IgG4-RLD are most likely distinct disease entities.

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“… 16–21 The protective effect of tacrolimus on CIDP has been reported in a few cases. 22–24 Therefore, we will assess the response of tacrolimus in patients with autoantibodies against paranodal proteins.…”

Section: Introductionmentioning

confidence: 99%

Tacrolimus Combined with Corticosteroids Improved the Outcome of CIDP Patients with Autoantibodies Against Paranodal Proteins

Yang¹,

Li²,

Ji³

et al. 2022

NDT

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Purpose To investigate the response of tacrolimus to chronic inflammatory demyelinating polyneuropathy (CIDP) with autoantibodies against paranodal proteins, including neurofascin-155 (NF155), contactin-1 (CNTN1) and contactin-associated protein 1 (Caspr1). Methods We retrospectively reviewed all CIDP patients who carried anti-NF155, CNTN1 and Caspr1 antibodies and were treated with tacrolimus at Tongji hospital from Jan 2018 to Apr 2021. Results There were 58 patients with CIDP and only 9 patients had autoantibodies against paranodal proteins (17.2%). Five of the 9 patients received tacrolimus treatment with an initial dose of 2–3 mg once daily. One patient with anti-CNTN1 antibody started tacrolimus and corticosteroid treatment, at the first episode and eventually achieved full clinical remission without relapse. Four patients with anti-NF155 or -Caspr1 antibodies experienced relapse during corticosteroids tapering. Then, they were given oral tacrolimus and presented with clinical improvement. During follow-up, only one patient developed worsening weakness due to unreasonable tacrolimus discontinuation. Moreover, 3 patients were successfully withdrawn from corticosteroids and 2 patients took corticosteroids at low maintenance dose (10mg/d) after tacrolimus treatment. No severe adverse events were observed in all the patients. Conclusion Patients with autoantibodies against paranodal proteins had a better long-term outcome after adding tacrolimus. Combination therapy with corticosteroids and tacrolimus may be an effective therapeutic regimen.

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Anti-Neurofascin–Associated Nephrotic-Range Proteinuria in Chronic Inflammatory Demyelinating Polyneuropathy

Cited by 13 publications

References 18 publications

Anti-neuronal IgG4 autoimmune diseases and IgG4-related diseases may not be part of the same spectrum: a comparative study

Anti-neuronal IgG4 autoimmune diseases and IgG4-related diseases may not be part of the same spectrum: a comparative study

Anti-Neuronal IgG4 Autoimmune Diseases and IgG4-Related Diseases May Not Be Part of the Same Spectrum: A Comparative Study

Tacrolimus Combined with Corticosteroids Improved the Outcome of CIDP Patients with Autoantibodies Against Paranodal Proteins

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