Tacrolimus Combined with Corticosteroids Improved the Outcome of CIDP Patients with Autoantibodies Against Paranodal Proteins

PURPOSE: To investigate the response of tacrolimus to chronic inflammatory demyelinating polyneuropathy (CIDP) with autoantibodies against paranodal proteins, including neurofascin-155 (NF155), contactin-1 (CNTN1) and contactin-associated protein 1 (Caspr1). METHODS: We retrospectively reviewed all CIDP patients who carried anti-NF155, CNTN1 and Caspr1 antibodies and were treated with tacrolimus at Tongji hospital from Jan 2018 to Apr 2021. RESULTS: There were 58 patients with CIDP and only 9 patients had autoantibodies against paranodal proteins (17.2%). Five of the 9 patients received tacrolimus treatment with an initial dose of 2–3 mg once daily. One patient with anti-CNTN1 antibody started tacrolimus and corticosteroid treatment, at the first episode and eventually achieved full clinical remission without relapse. Four patients with anti-NF155 or -Caspr1 antibodies experienced relapse during corticosteroids tapering. Then, they were given oral tacrolimus and presented with clinical improvement. During follow-up, only one patient developed worsening weakness due to unreasonable tacrolimus discontinuation. Moreover, 3 patients were successfully withdrawn from corticosteroids and 2 patients took corticosteroids at low maintenance dose (10mg/d) after tacrolimus treatment. No severe adverse events were observed in all the patients. CONCLUSION: Patients with autoantibodies against paranodal proteins had a better long-term outcome after adding tacrolimus. Combination therapy with corticosteroids and tacrolimus may be an effective therapeutic regimen.