“Neuro‐renal syndrome” related to anti‐contactin‐1 antibodies

Guillaume Taieb (1) , Moglie Le Quintrec (2, 3) , Amandine Pialot , Ilan Szwarc (4) , Hélène Perrochia (2) , Pierre Labauge (5) , Jérôme Devaux (6)

1 Département de neurologie [Montpellier]
2 CHRU Montpellier - Centre Hospitalier Régional Universitaire [Montpellier]
3 Cellules Souches, Plasticité Cellulaire, Médecine Régénératrice et Immunothérapies (IRMB)
4 NDSG - Néphrologie Dialyse Saint Guilhem
5 Service de Neurologie
6 INM - Institut des Neurosciences de Montpellier

Since 1973, a few cases of concomitant inflammatory demyelinating polyradiculoneuropathy and nephrotic syndrome have been reported. The neuropathy was either an acute inflammatory demyelinating polyneuropathy (AIDP) or a chronic inflammatory demyelinating polyneuropathy (CIDP), and the nephrotic syndrome was related to either a focal segmental glomerulosclerosis (FSGS) or a membranous glomerulonephritis (MGN). Time course, severity, and response to treatment were heterogeneous, and evolution of neuropathy and nephropathy could be independent. This suggests that common epitopes are targeted by autoantibodies within the peripheral nerve and the glomerular filtration barrier. However, the antigenic targets are unknown. Here, we report a patient who developed CIDP associated with anti-contactin-1 immunoglobulin G4 (IgG4) antibodies and nephrotic syndrome.

Mots clés: anti‐contactin‐1 IgG4 antibodies; anti‐neurofascin‐186 IgG4 antibodies; chronic inflammatory demyelinating polyradiculoneuropathy; nephrotic syndrome; nodopathy; paranodopathy

Domaines: Sciences du Vivant [q-bio]; Neurobiologie; Immunité adaptative; Interactions cellulaires [q-bio.CB]; Neurosciences [q-bio.NC]

Guillaume Taieb, Moglie Le Quintrec, Amandine Pialot, Ilan Szwarc, Hélène Perrochia, et al.. “Neuro‐renal syndrome” related to anti‐contactin‐1 antibodies. Muscle & nerve. Supplement., 2019, 59 (3), ⟨10.1002/mus.26392⟩. ⟨hal-02347676⟩