Autoimmune Nodopathy (AN) has been classified as an independent disease due to significant differences in pathological features and clinical manifestations from chronic inflammatory demyelinating polyradiculoneuropathy (CIDP), yet it is often overlooked or misdiagnosed. We report a case of neurofascin-155 (NF155) + AN in a 76-year-old male. Innovatively, we introduced the dual B-lymphocyte stimulator (BLyS) and a proliferation-inducing ligand (APRIL) inhibitor telitacicept as first-line therapy immediately after diagnosis. This therapeutic approach led to rapid and sustained disease control in the patient. Beyond demonstrating the critical importance of early diagnosis and targeted therapy in preventing irreversible neurological damage, this case provides compelling preliminary evidence for the favorable efficacy and safety of telitacicept, supporting its potential as a first-line treatment for AN, particularly in the NF155 + subtype.
Luan et al. (Tue,) studied this question.