Validation of Cutaneous Nerve Demyelination in Diagnosis and Treatment of CIDP

Eligibility Criteria

• * Adults (age ≥ 18 years) with definite or probable CIDP according to the EFNS/PNS criteria may enter the trial within 12 months of treatment onset or treatment naïve patients. Written informed consent is obtained by the local investigator before entry into the study.
• 2. IVIg treatment of 0.8 up to 1.2 g/kg per 30 days will be allowed up to 6 months. It is expected some patients may be on variable treatment regimens, and as along as they are not significantly declining, efforts will be made to continue treatment regimens already instituted.
• 3. Prednisone in doses up to 20 mg /day will be allowed as long as dose has been stable for 90 days and is not being escalated or tapered during study.
• 4. Previous plasma exchange will be allowed as long as it is not continued during the study.
• * 1. Other causes of polyneuropathy, multifocal motor neuropathy, diabetes mellitus, alcohol, family history of neuropathy, monoclonal gammopathy or malignancy, history of drug or toxin exposure, which could reasonably cause neuropathy. Coexistent monoclonal gammopathy will also be used as an exclusion as these patients may not have the same response to IVIg.
• 2. Any other disease that may cause neurological symptoms and signs or that may interfere with treatment or outcome assessments.
• 3. Severe conditions that may interfere with an evaluation of the study product or satisfactory conduct of the study such as current malignancy or history of allogeneic bone marrow/stem cell transplant, cardiac insufficiency (New York Heart Association Classes III/IV), cardiomyopathy, cardiac arrhythmia requiring treatment, unstable or advanced ischemic heart disease, congestive heart failure or severe hypertension, chronic kidney disease deemed too severe to safely use IVIg, known hyperprolinemia, known bleeding disorders, severe skin disease at the planned injection sites or biopsy site, alcohol, drug or medication abuse.
• 4. History of keloids or other reactions to local anesthetic making skin biopsies unsafe.
• 5. Patients with the following laboratory results:
• 1. Positive result at screening on any of the following viral markers: human immunodeficiency virus-1 or 2, or hepatitis B or C virus.
• 2. Abnormal laboratory parameters: creatinine greater than 1.5 times the upper limit of normal (ULN), blood urea nitrogen greater than three times the ULN if the increase is related to potential kidney disease, or hemoglobin less than 10 g/dL
• 7. Additional medications and treatments other than prednisone and IVIg for treatment of CIDP such as azathioprine, mycophenolate, rituximab and ongoing plasma exchange as this will either confound or interfere with effects of IVIg.