While it is the most common chronic autoimmune neuropathy, chronic inflammatory demyelinating polyneuropathy (CIDP) is a rare, acquired peripheral neuropathy characterised by proximal upper and lower limb weakness. CIDP remains a challenging diagnosis with a poorly understood disease mechanism.1,2 The current standard of care involves corticosteroids, intravenous immunoglobulin (IVIg) or plasmapheresis. Different studies relating to CIDP were presented at AAN in this CIDP Awareness Month; key findings of two posters are summarised below:
Islam (Rutgers University, USA) and colleagues investigated the association of four autoimmune disorders with CIDP compared to multiple sclerosis (MS) and migraine in an attempt to unveil similarities in the underlying disease mechanisms.2 A significantly higher incidence of combined autoimmune disorders was observed in individuals with CIDP compared to both MS and migraine (13.1%, 0.69% and 0.45%, respectively; p < 0.0001). Guillain-Barré syndrome was most frequently reported in people with CIDP compared to MS and migraine (11.2%, 0.11% and 0.03%, respectively; p < 0.0001), followed by myasthenia gravis (0.88%, 0.21% and 0.04%, respectively; p < 0.001) and systemic lupus erythematosus (0.74%, 0.35% and 0.37%, respectively; p < 0.0015). No significant difference in the incidence of acute disseminated encephalomyelitis was observed in individuals with CIDP compared to MS and migraine (0.07%, 0.02% and 0.0007%, respectively; p = 0.10). The authors concluded that the association observed between CIDP and other autoimmune disorders can lead to improved understanding of the diagnosis and treatment of CIDP.
The extent of disease-related disability of CIDP and the potential impact of its treatment on patient-reported outcomes was investigated by Allen (University of Minnesota, USA) and colleagues with a US nationwide online patient survey.3 Most survey participants reported experiencing nine symptoms or more, with almost 50% selecting all of the ten listed symptoms. Loss of balance/coordination, weakness in the hips/legs and numbness of hands or feet were among the most bothersome symptoms reported. The impact on work is substantial – 47% of participants reported having to stop working due to the disease, with another 14% having to change jobs or reduce working hours. The most common treatments, corticosteroids and IVIg, are most frequently discontinued because of poor tolerability and limited symptom improvement. These survey results highlight not only the large burden of disease-related disability on individuals with CIDP, but that of its current treatments as well.
With case reports emerging of CIDP in previously unreported diseases4, will this common autoimmune neuropathy become the next major challenge in tackling autoimmune disorders?
- M C Dalakas. “Advances in the diagnosis, pathogenesis and treatment of CIDP”. Nat Rev Neurol. 2011:7;507–17.
- S Islam, et al. Does CIDP Increase The Risk of Acquiring Other Autoimmune Disorders? A New York State Planning and Research Cooperation System Database Analysis (1998-2014). Poster presented at: AAN Annual Meeting 2019; May 6, 2019; Philadelphia, PA, USA. Abstract P2.2-105.
- J A Allen, et al. CIDP Disease Burden: Results of a US Nationwide Patient Survey. Poster presented at: AAN Annual Meeting 2019; May 6, 2019; Philadelphia, PA, USA. Abstract P2.2-104.
- M Murthy and M Hillen. First report of confirmed Chronic Inflammatory Demyelinating Polyneuropathy (CIDP) in NMO Spectrum Disorder (NMOSD). Poster presented at: AAN Annual Meeting 2019; May 6, 2019; Philadelphia, PA, USA. Abstract P2.2-106.