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In a recent analysis, patients with chronic inflammatory demyelinating polyneuropathy who started their initial treatment later experienced a worsening of the disease course.
Tobias Ruck, MD, PhD
(Credit: Heinrich Heine University Düsseldorf)
In a newly published retrospective analysis in the Journal of Neurology, findings revealed potential clinical factors that influence the disease progression and overall treatment efficacy among patients with chronic inflammatory demyelinating polyneuropathy (CIDP). Overall, the results suggested that having a timely diagnosis, prompting the initiation of treatment, and careful monitoring of patients’ treatment response are essentials for the prevention of long-term disability in CIDP.1
In a cohort of 87 patients with CIDP that received immunomodulatory therapy, most were on intravenous immunoglobulins or prednisolone. Overall, those who responded to their first therapy (n = 110, 56%) were considered a Standard of Care (SOC)-responder and patients who did not (n = 76, 39%) were defined as SOC-refractory. Most of the SOC-responders received immunoglobulins (n = 67, 61%) or prednisolone (n = 38, 35%) as their initial therapy. Notably, 35 patients of the SOC-refractory cohort (46%) received immunoglobulins and 32 (42%) received prednisolone.
Senior author Tobias Ruck, MD, PhD, deputy director of the department of neurology at Heinrich Heine University Düsseldorf in Germany, and colleagues wrote, “Our research highlights the urgent need for advances in the understanding of CIDP, including its risk factors, pathophysiology and therapeutic approaches, and describes the current knowledge gaps that require further investigation and research. We focused on the clinical deterioration of CIDP patients by extending the definition of SOC-responder patients to sustained SOC-responders or transitioned SOC-refractory, respectively.”1
In this analysis, investigators screened 197 patients with CIDP who were presented to the University Hospitals in Düsseldorf, Berlin, Cologne, Essen, Magdeburg, and Munich between 2018 and 2022. Researchers then used the respective hospital information system and investigated the baseline data with clinical examination, medical letters, laboratory results, antibody status, nerve conduction studies, imaging and biopsy results. In addition to baseline data, authors examined treatment outcomes utilizing the SOC definition, as well as a comparison of an early—in the first 12 months after manifestation—versus late—more than 12 months after manifestation—onset of the therapy.
READ MORE: Case Study Shows Efficacy of BCMA-CD19 Bispecific CAR-T Therapy in Refractory CIDP
In further analysis, the Inflammatory Neuropathy Cause and Treatment (INCAT)2 arm disability scores of patients with early disease onset displayed a significant (P <.01) reduction 36 months following diagnosis, as well as 24 months after diagnosis (P <.05), between 12 and 24 months (P <.05), 12 and 36 months (P <.001), and between 24 and 36 months (P <.01) after diagnosis.
Authors observed that the INCAT leg disability scores of patients with a late treatment start significantly (P < 0.01) lowered at diagnosis and had a significant increase 24 (P <.05) and 36 (P <.01) months following diagnosis, as well as between 12 and 24 (P <.05), and between 12 and 36 (P <.01) months after diagnosis.
The INCAT arm disability scores of SOC-responder patients revealed a significant decrease at 12 (P <.001), 24 (P <.01) and 36 (P <.05) months following diagnosis. In contrast, the INCAT leg disability score of SOC-refractory identified patients significantly increased 24 and 36 months (P <.05) after diagnosis, as well as between 12 and 24 months (P <.01), 24 and 36 months (P <.01), and between 24 and 36 months (P <.05) following diagnosis. Additionally, the INCAT leg disability score of SOC-responder defined patients 36 months after diagnosis was significantly (P < 0.05) lowered in comparison with the SOC-refractory group.
“Regular monitoring of treatment response should be integrated more frequently into clinical routine in order to allow treatment changes in time. Additionally, we could point out the importance of an early diagnosis and start of treatment to halt lasting disability favoring a hit hard and early treatment strategy,” Ruck et al noted.1 “However, the complexity of clinical management of CIDP remains as the lack of reliable biomarkers capable of indicating clinical disease activity and identifying patients at risk of disease worsening continues to impede the integration of effective clinical practice. Hence, there is an urgent need for prospective clinical and molecular tools to advance the diagnosis and management of CIDP.”
