Long-term follow-up of relapse and remission of CIDP in a Chinese cohort

OBJECTIVE: We aim to describe the long-term outcome of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) after immune treatment in a Chinese cohort.

METHODS: Between March 2015 and March 2023, 89 patients fulfilling the criteria for CIDP were followed up for a median of 22 months after treatment.

Nine had positive antibodies against nodal-paranodal cell-adhesion molecules.

Patients were treated according to clinical requirements with prednisone, intravenous immunoglobulin (IVIg) and/or immunosuppressant.

RESULTS: A total of 78/89 patients had decreased inflammatory neuropathy cause and treatment (INCAT) scores at the last follow-up.

For CIDP patients treated with steroids, 35 were stable without relapse after cessation or with a small maintenance dose; 2 relapsed at a high dose (20 mg/day); 15 relapsed at a low dosage (<20 mg/day) and 11 did not respond.

The INCAT before treatment was significantly lower in those without relapse (median INCAT 2 vs 3, p=0.030).

IVIg was effective in 37/52 CIDP patients.

28 CIDP patients and 4 autoimmune nodopathy patients were treated with immunosuppressants.

The average INCAT was 3.3±1.9 before and 1.9±1.3 after immunosuppressant treatment (p=0.001) in CIDP.

CONCLUSION: The long-term prognosis of CIDP patients was generally favourable.

Nearly half of our patients treated with steroid were stable without relapse after cessation or with a small maintenance dose.

The risk of relapse was higher in those with high INCAT.

We recommend slowly tapering prednisone based on clinical judgement.