Background and Aims

Few case reports/series describe the efficacy of rituximab in refractory CIDP, which is preferred in the presence of anti-nodal/paranodal antibodies. We aimed at evaluating the clinical response to rituximab in a subset of patients with refractory CIDP for whom anti-nodal/paranodal antibodies status was unknown, as not available in Iran.

Methods

We retrospectively analyzed the response to rituximab in 14 Iranian patients with refractory CIDP (3 children, 11 adults), in whom the anti-nodal/paranodal antibodies status was unknown. Subjects were evaluated with the Medical Research Council (MRC) sum score (MRCSS), Inflammatory Neuropathy Cause and Treatment (INCAT) disability scores, and electrophysiology, before and after treatment.

Results

Mean age was 34.4 ± 20.7 years, disease duration pre-rituximab treatment was 27.8 ± 18.8 (range: 6 - 60) months, and mean follow-up duration was 18.5 ± 11.0 (range: 4 – 36) months. Considering INCAT sumscore, one worsened during post-rituximab treatment, and three patients did not change. Considering MRCSS, notably, four patients achieved normalization of their MRCSS. Regarding corticosteroid dose, two patients could discontinue prednisolone.

As rated by a pre-defined scoring system, nerve conduction parameters improved significantly post-rituximab in the treated cohort (p=0.006). All patients tolerated rituximab infusions without adverse effects.

Interpretation

Rituximab may be effective in refractory CIDP, even though worsening may occur in some patients. Anti-nodal/paranodal antibodies assay, when available, and other criteria may help drive therapeutic decision-making on rituximab as second-line treatment.

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