Dataset Information

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

ABSTRACT: There are few case reports of concomitant chronic inflammatory demyelinating polyneuropathy (CIDP) and focal segmental glomerulosclerosis. A rare autoantibody to a neuronal and podocyte structural component, neurofascin, may be contributory. A Black man in his 40s presented with worsening polyneuropathy requiring mechanical ventilation and initially acute inflammatory demyelinating polyneuropathy was diagnosed. After a poor response to intravenous immunoglobulin, plasmapheresis was initiated. The patient also had concomitant new-onset nephrotic-range proteinuria. A limited kidney biopsy was interpreted as minimal change disease and was treated with prednisone. After some improvement, the patient was extubated; however, he later re-presented with worsening symptoms requiring mechanical ventilation and was re-treated with plasmapheresis. Due to the protracted course and poor response to intravenous immunoglobulin, acute-onset CIDP was diagnosed and a neuromuscular antibody workup returned positive for neurofascin, supporting the diagnosis of seropositive acute-onset CIDP. A repeat kidney biopsy demonstrated focal segmental glomerulosclerosis and acute tubular damage. The patient was treated with steroids and tacrolimus and later transitioned to rituximab. Neurofascin enzyme-linked immunosorbent assay then tested negative with concomitant resolution of both neuropathy and proteinuria. Further studies will help validate these findings and the treatment strategy.

SUBMITTER: Bukhari S

PROVIDER: S-EPMC7729218 | biostudies-literature | 2020 Nov-Dec

REPOSITORIES: biostudies-literature

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

Bukhari Syed S Bettin Margaret M Cathro Helen P HP Gwathmey Kelly K Gautam Jitendra J Bowman Brendan B

Kidney medicine 20201023 6

There are few case reports of concomitant chronic inflammatory demyelinating polyneuropathy (CIDP) and focal segmental glomerulosclerosis. A rare autoantibody to a neuronal and podocyte structural component, neurofascin, may be contributory. A Black man in his 40s presented with worsening polyneuropathy requiring mechanical ventilation and initially acute inflammatory demyelinating polyneuropathy was diagnosed. After a poor response to intravenous immunoglobulin, plasmapheresis was initiated. Th ...[more]

PMID: 33319203

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Project description:BackgroundRefractory chronic inflammatory demyelinating polyneuropathy (CIDP) is a challenging subset of CIDP. It does not respond well to immune therapy and causes substantial disability. A comprehensive understanding of its clinical profile, electrophysiological characteristics and potential risk factors associated with refractoriness remains to be further elucidated.MethodsData in this cross-sectional study was collected and reviewed from the Huashan Peripheral Neuropathy Database (HSPN). Included patients were categorized into refractory CIDP and non-refractory CIDP groups based on treatment response. The clinical and electrophysiological characteristics were compared between refractory and non-refractory CIDP groups. Potential risk factors associated with refractory CIDP were explored with a multivariate logistic regression model.ResultsFifty-eight patients with CIDP were included. Four disease course patterns of refractory CIDP are described: a relapsing-remitting form, a stable form, a secondary progressive form and a primary progressive form. Compared to non-refractory CIDP patients, refractory CIDP exhibited a longer disease duration (48.96 ± 33.72 vs. 28.33 ± 13.72 months, p = 0.038) and worse functional impairment (MRC sum score, 46.08 ± 12.69 vs. 52.81 ± 7.34, p = 0.018; mRS, 2.76 ± 0.93 vs. 2.33 ± 0.99, p = 0.082; INCAT, 3.68 ± 1.76 vs. 3.03 ± 2.28, p = 0.056, respectively). Electrophysiological studies further revealed greater axonal impairment (4.15 ± 2.0 vs. 5.94 ± 2.77 mv, p = 0.011, ulnar CMAP) and more severe demyelination (5.56 ± 2.86 vs. 4.18 ± 3.71 ms, p = 0.008, ulnar distal latency, 7.94 ± 5.62 vs. 6.52 ± 6.64 ms, p = 0.035, median distal latency; 30.21 ± 12.59 vs. 37.48 ± 12.44 m/s, p = 0.035, median conduction velocity; 58.66 ± 25.73 vs. 42.30 ± 13.77 ms, p = 0.033, median F-wave latency), compared to non-refractory CIDP. Disease duration was shown to be an independent risk factor for refractory CIDP (p < 0.05, 95%CI [0.007, 0.076]).ConclusionThis study provided a comprehensive description of refractory CIDP, addressing its clinical features, classification of clinical course, electrophysiological characteristics, and prognostic factors, effectively elucidating its various aspects. These findings contribute to a better understanding of this challenging subset of CIDP and might be informative for management and treatment strategies.

Dataset Information

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

Publications

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

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