Anti-Neurofascin–Associated Nephrotic-Range Proteinuria in Chronic Inflammatory Demyelinating Polyneuropathy
| Autor: | Helen P. Cathro, Syed Nasir Abbas Bukhari, Jitendra Gautam, Brendan T. Bowman, Kelly G. Gwathmey, Margaret Bettin |
|---|---|
| Rok vydání: | 2020 |
| Předmět: |
medicine.medical_specialty
medicine.medical_treatment Case Report Chronic inflammatory demyelinating polyneuropathy CIDP lcsh:RC870-923 Gastroenterology Focal segmental glomerulosclerosis Prednisone Internal medicine Internal Medicine medicine Minimal change disease demyelinating polyneuropathy nephrotic syndrome business.industry lcsh:Diseases of the genitourinary system. Urology medicine.disease FSGS Nephrology Rituximab Plasmapheresis anti-neurofascin proteinuria business Nephrotic syndrome Polyneuropathy medicine.drug |
| Zdroj: | Kidney Medicine, Vol 2, Iss 6, Pp 797-800 (2020) Kidney Medicine |
| ISSN: | 2590-0595 |
| DOI: | 10.1016/j.xkme.2020.06.016 |
| Popis: | 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. |
| Databáze: | OpenAIRE |
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