Clinical Characteristics and Treatment Response to Rituximab in Refractory Chronic Inflammatory Demyelinating Polyradiculoneuropathy: A Retrospective Observational Study from a Single Tertiary Center in Thailand
Chaichana Sinthuwong*, Metha Apiwattanakul, Narupat Suanprasert and Saharat Aungsumart
Department of Neurology, Neurological Institute of Thailand, Bangkok, Thailand
*Corresponding Author: Chaichana Sinthuwong, Department of Neurology, Neurological Institute of Thailand, Bangkok, Thailand.
Received:
June 05, 2024; Published: July 04, 2024
Abstract
Background: Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is a disabling condition requiring immunosuppressive or immunomodulatory drug treatment. However, approximately 20% of patients do not respond to classical immunosuppressive drugs. Rituximab (RTX) is beneficial for patients with refractory CIDP.
Objective: This study aimed to evaluate the efficacy of RTX in treating refractory CIDP.
Methods: This retrospective study evaluated 11 patients with refractory CIDP who were treated with RTX. The Medical Research Council (MRC) sum score, Inflammatory Neuropathy Cause and Treatment (INCAT) disability scale, and Modified Rankin Scale (MRS) were analyzed at 12 and 24 weeks after RTX treatment compared to baseline.
Results: The main clinical characteristics of refractory CIDP were distal weakness predominance (54.5%), accompanied by tremors (54.5%), and sural nerve pathology without onion bulb formation (100%). At 12 and 24 weeks post-RTX treatment, the median MRC sum score improved from 46 (interquartile range [IQR] 36-56) to 50 (IQR 48-58, p-value = 0.005) and 58 (IQR 52-60, p-value = 0.008), respectively. The median INCAT disability scale score improved from 6 (IQR 6-7) to 5 (IQR 4-6, p-value = 0.006) and 3 (IQR 1-5, p-value = 0.004), respectively. The median MRS sum score improved from 6 (IQR 6-7) to 3 (IQR 2-4, p-value <0.016) and 3 (IQR 1-3, p-value < 0.003), respectively.
Conclusion: RTX was effective in treating patients with refractory CIDP. Clinical features like distal weakness, tremors, and nerve pathology without onion bulb formation may prompt clinicians to consider refractory CIDP as a diagnosis.
Keywords: Chronic Inflammatory Demyelinating Polyradiculoneuropathy (CIDP); Refractory CIDP; Rituximab; Nodal and Paranodal Polyneuropathies; Inflammatory Neuropathy Cause; Treatment (INCAT)
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