Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is a heterogeneous immune-mediated neuropathy with up to 30% of such patients refractory to standard treatments. Rituximab, a B-cell-depleting monoclonal antibody, has shown promising efficacy in antibody-positive autoimmune diseases, but its efficacy in seronegative CIDP remains uncertain. We report the case of a 46-year-old man with severe refractory seronegative CIDP who experienced a rapid deterioration two weeks after rituximab initiation, while developing tetraplegia and respiratory failure that required mechanical ventilation. The patient subsequently achieved a full recovery and sustained remission for over 2.5 years. This highlights the therapeutic potential of rituximab in seronegative refractory CIDP, warranting further studies to identify the responsive subtypes and underlying mechanisms.
Okubo et al. (Thu,) studied this question.