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

Abstract

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 Moreover, 3 patients were successfully withdrawn from corticosteroids and 2 patients took corticosteroids at low maintenance dose (10mg/d) after tacrolimus treatment.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.

Autoantibodies against contactin‐associated protein 1 and complexes of paranode‐specific proteins in chronic inflammatory demyelinating polyradiculoneuropathy

“…This is the first Japanese case of anti‐Caspr1 antibody‐positive CIDP, and the clinical characteristics of this case were similar to those of other cases reported from Western countries, including subacute onset, distal‐predominant limb weakness, sensory ataxia, postural hand tremor, marked increased in cerebrospinal fluid protein levels, electrophysiologically multifocal demyelinating conduction disturbance, and inadequate response to transvenous immunoglobulin therapy and steroids 5,6,11,13 . Interestingly, previous studies have suggested that anti‐Caspr1 antibody‐positive CIDP is also characterized by neuropathic pain, cranial nerve involvement or respiratory failure 5,6,11,13,18–20 ; however, the present patient showed none of these. These findings indicate that the clinical signature of Caspr1‐positive cases is similar to that of other paranode antibody‐positive cases, although there are slight differences in the clinical presentation from case to case.…”