Challenges in the diagnosis and treatment of chronic inflammatory demyelinating polyneuropathy

Chronic inflammatory demyelinating polyneuropathy (CIDP) consists of a spectrum of autoimmune diseases of the peripheral nerves with a very heterogeneous clinical presentation. Different pathophysiological mechanisms have been identified, not always sharing the same clinical features, which makes both diagnosis and choosing right treatment strategy challenging. Diagnosis is based on consensus guidelines, as no golden standard test is available. The aim of this thesis was to identify some of the challenges in treatment and diagnosis. In this thesis, we found a new nerve conduction study strategy to diagnose asymmetric CIDP. Additionally, we found that an elevated leukocyte count in the cerebrospinal fluid, does not exclude the diagnosis, even though the diagnostic criteria use a cut-off value of 10 cells/ul. We also identified patients who did not meet the mandatory diagnostic criteria but did respond to immunomodulatory treatment. Lastly, we conducted a randomized controlled non-inferiority trial, in which we compared IVIg withdrawal with placebo with continuation of IVIg treatment. In this study we showed that an important part of patients can stop treatment and that IVIg withdrawal is safe.