In the very rare patients with UCTD and CNS demyelination, usually reported in case reports, the CNS demyelination is in the form of myelitis (“lupus myelitis”), or has been part of neuromyelitis optica (NMO) or MOG-associated disease (MOGAD) [7, 8]; the patient in this report had negative aquaporin-4 and MOG antibodies and did not have a clinical course consistent with NMO or MOGAD. Here, AQP4 is linked to CNS demyelination.