Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is a
disabling autoimmune disorder of the peripheral nervous system (PNS).
Intravenous immunoglobulins (IVIg) are effective in CIDP, but the treatment
response varies greatly between individual patients. Understanding this
interindividual variability and predicting the response to IVIg constitute
major clinical challenges in CIDP. We previously established intercellular
adhesion molecule (ICAM)-1 deficient non-obese diabetic (NOD) mice as a novel
animal model of CIDP. Here, we demonstrate that similar to human CIDP
patients, ICAM-1 deficient NOD mice respond to IVIg treatment by clinical and
histological measures. Nerve magnetic resonance imaging and histology
demonstrated that IVIg ameliorates abnormalities preferentially in distal
parts of the sciatic nerve branches. The IVIg treatment response also featured
great heterogeneity allowing us to identify IVIg responders and non-
responders. An increased production of interleukin (IL)-17 positively
predicted IVIg treatment responses. In human sural nerve biopsy sections, high
numbers of IL-17 producing cells were associated with younger age and shorter
disease duration. Thus, our novel animal model can be utilized to identify
prognostic markers of treatment responses in chronic inflammatory neuropathies
and we identify IL-17 production as one potential such prognostic marker
