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Recurrent Neuro-Renal Syndrome With Acute Kidney Injury From Anti-Pan-Neurofascin Antibody Resurgence: A Case Report
David De Saint Gilles1,2, Cédric Rafat1,2, Jérôme Joël Devaux3
1Soins Intensifs Néphrologiques et Rein Aigu (SINRA), Assistance publique - Hôpitaux de Paris, Hôpital Tenon, Paris, France.
Abstract:
We report the case of a patient with a severe neuro-renal syndrome characterized by acute-onset autoimmune nodopathy and nephrotic syndrome with acute kidney injury. The presence of IgG3 antibodies anti-pan-neurofascin (Nfasc155/186) confirmed the autoimmune nature of this pathology and justified treatment with plasma exchange, corticosteroids, tacrolimus, and subsequently rituximab. Kidney biopsy revealed focal segmental glomerulosclerosis lesions. This therapeutic approach led to complete remission of the clinical presentation. APOL1 genotyping revealed G1/G1 alleles. One year after discontinuation of maintenance therapy with corticosteroids and tacrolimus, the patient experienced a relapse of the neuro-renal syndrome. This relapse was concomitant with the reappearance of anti-pan-neurofascin antibodies, which were undetectable during the first remission. Resumption of plasma exchange therapy guided by decreasing antibody titers along with corticosteroids and tacrolimus enabled neurologic remission, although chronic kidney disease persisted. Maintenance therapy with rituximab was initiated to prevent further relapses. This first reported case of late recurrence of anti-pan-neurofascin-associated neuro-renal syndrome highlights the importance of comprehensive immunologic and genetic evaluation, close immunomonitoring, and targeted immunotherapies, including plasma exchange and long-term rituximab maintenance.
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