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. 2025 Feb 5;12(2):e200369. doi: 10.1212/NXI.0000000000200369

Table.

Patients With CIPO Treated With Rituximab or Vedolizumab

First author (ref) Age/sex Additional neurological symptoms Antibody Intestinal pathologic examination Cancer Failed immunotherapies Second-line drug and response
Coret12 60/F Sensory neuronopathy Hu Not done None Corticosteroids, IVIG, PLEX Rituximab. CIPO: complete response; sensory neuronopathy: partial response
Badari13 61/M No Hu Diffuse lymphoplasmacytic infiltration of myenteric plexus SCLC IVIG, octreotide Rituximab. Complete response (concomitant treatment with cyclophosphamide)
Drukker14 4/M Sensory neuropathy Hu Lack of ganglion cells in myenteric plexus Neuroblastoma Prednisone Rituximab. No responsea
Schamphelaere15 58/M Brainstem and limbic encephalitis Hu T-cell infiltration of myenteric plexus None Prednisone and PLEX Rituximab. No response (concomitant treatment with cyclophosphamide)
Vilaseca (patient 1) 45/M Myasthenia gravis None CD8+ T cells myenteric ganglionitis, reduction of ganglionic neurons Thymoma Prednisone and azathioprine Vedolizumab and low-dose prednisone. Clinical and radiologic response after 12 mo of follow-up
Vilaseca (patient 2) 7/M Sensory neuropathy and dysautonomia Hu None IVIg Vedolizumab. No response
Vilaseca (patient 3) 19/F No Hu CD8+ T cells myenteric ganglionitis, reduction of ganglionic neurons None Prednisone, adalimumab Methylprednisolone, IVIg, rituximab. Clinical and radiologic response after 12 mo of follow-up
Vilaseca (patient 4) 66/F No None T-cell infiltration myenteric ganglionitis None Prednisone Vedolizumab. Clinical and radiologic response after 12 mo of follow-up

Abbreviations: CIPO = Chronic Intestinal Pseudo-obstruction; IVIg = IV immunoglobulins; PLEX = plasma exchange; SCLC = small cell lung cancer.

a

PLEX induced a CIPO remission that was initially maintained by rituximab. Despite this treatment there were 4 relapses over the ensuing 17 months.