Study of CIDP Patients During IVIG Treatment
Condition: Polyradiculoneuropathy, Chronic Inflammatory Demyelinating
MeSH terms: Polyradiculoneuropathy; Polyradiculoneuropathy, Chronic Inflammatory Demyelinating
Study type: Observational

Summary: 
      The aim of this study is to quantify the effect of IVIG treatment in a group of patients with
      chronic inflammatory demyelinating polyradiculoneuropathy(CIDP), who requires continues
      treatment of IVIG at regular intervals of 3-10 weeks:

        1. During continues treatment of IVIG at regular intervals of 3-10 weeks.

        2. During pause in treatment.

      Hypothesis:

        1. The disease activity in the patients are cyclical correlating to the treatment
           intervals.

        2. Pause in treatment will increase disease activity, which can be quantified with symptom
           scores, disability scales, and clinical test.

      Primary effect parameter is muscle strength quantified by isokinetic dynamometry.

      Added to the protocol there is an immunological study of inflammatory markers in blood
      samples of patients under treatment pause.
    

Detailed description: 
      Background: Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is a rare
      autoimmune disease of the peripheral nervous system characterized by demyelination of the
      nerves, which causes muscle weakness and sensory loss. Treatment is immune modulating, and
      intravenous immunoglobulin (IVIG) is first line therapy.Several trials have demonstrated
      effect on motor function by the initial treatment, but the effect of consecutive IVIG
      treatment is only sporadic described in the literature.

      It is a clinical study including present CIDP patients in treatment at the University
      Hospital of Aarhus. The patients will be evaluated several times before and after IVIG
      treatment, to describe the effect profile.

      The primary effect parameter is muscle strength quantified by isokinetic dynamometry at ankle
      knee, hip, wrist, elbow and shoulder. That is a sensitive method of measuring the strength of
      the larger muscle groups, correlating with symptoms and signs of neuropathy.

      Severity of neuropathy among the patients will also be described applying nerve conduction
      studies, quantitative sensory testing of threshold for detecting vibration and cold at upper
      and lower limbs, the Neuropathy Disability Scale, the Neuropathy Symptom Score,the overall
      disability sum score, 9 hole peg test, walking test, and the Short-form 36 health
      questionnaire.

      Added to the protocol there is an immunological study of inflammatory markers in blood
      samples of patients under treatment pause.

      Objective:

      With this study we will describe some important aspects in the immune response causing the
      inflammatory lesions in CIDP and MMN, including:

        1. Recruitment of immune cells to the affected tissue by chemoattraction. (Chemokine
           receptors on mononuclear cells)

        2. Crossing the blood-nerve barrier: interactions and adhesion between the lymphocyte and
           endothelial cell, transendothelial diapedesis and enzymatic degeneration of the basal
           lamina.(Adhesion molecules on mononuclear cells and soluble in plasma,
           metalloproteinases)

        3. Synthesis of mRNA and secretion of regulatory cytokines.
    

Intervention name(s): intravenous gammaglobulin
Intervention type(s): Drug
Intervention description(s): individual dose
Intervention MeSH terms: gamma-Globulins
Time perspective: Prospective
Keywords: CIDP; IVIG; gammaglobulin; Chronic Inflammatory Demyelinating Polyradiculoneuropathy