Although clinical research in chronic inflammatory demyelinating polyneuropathy (CIDP) has addressed important issues in the management of patients with CIDP, there is still a potpourri of unresolved questions. These include foremost diagnosis and treatment, as well as related aspects such as epidemiology and economic burden of the disease. As an industry-leading biopharmaceutical company focusing on the therapeutic development of autoimmune disease, Creative Biolabs has paid a lot of manpower, material, and financial resources in relative research and thus established a comprehensive platform to provide global researchers with satisfying services to accelerate the progress of this field.
CIDP is the term used for a group of disorders that are thought to be immune-mediated. They all are, by definition, chronically progressive or relapsing for over 8 weeks. They have electrophysiologic and/or pathologic findings of segmental demyelination and respond to anti-inflammatory, immune-modulating, or immunosuppressive treatments. The cause of CIDP is unknown, although abnormalities in both cellular and humoral immunity have been shown. Until recently, no form of CIDP has been confirmed that it is associated with a specific antigen/antibody.
Clinically, CIDP has been classified into 'typical' and 'atypical' cases. Typical CIDP is defined as being a symmetric motor/sensory disorder with proximal and distal weakness and areflexia with conduction slowing, temporal dispersion, and/or conduction block. Atypical cases include pure sensory or sensory predominant cases, multifocal cases with persistent conduction block (Lewis Sumner Syndrome), and cases with symmetric distal signs and symptoms.
Fig.1 Immunopathogenesis of chronic inflammatory demyelinating polyneuropathy. (Mathey, 2015)
Treatment of patients with CIDP is complex and requires individualized treatment strategies. First-line therapies that are efficacious include corticosteroids, intravenous immunoglobulin (IVIG), and plasmapheresis.
One of the hallmarks of CIDP is that it responds to corticosteroid treatment. An early randomized controlled trial demonstrated that patients who received prednisone at an equivalent of 60 mg a day, tapered over three months, resulted in a better neuropathy disability score (NDS), later renamed neuropathy impairment score (NIS), compared with placebo. Subsequently, various investigators reported that pulsed steroid regimens are also effective while causing relatively fewer side effects compared with daily oral steroids.
Randomized controlled studies have demonstrated that CIDP patients who received an initial treatment of IVIG could have improvement of their strength and disability scores within 2 to 4 weeks of the infusion. It was also shown that relapsing symptoms may occur from 3 to 22 weeks after an initial treatment of IVIG at 2 g/kg, and additional IVIG can be used to maintain the benefit of the initial treatment. For patients who respond to IVIG but prefer more autonomy, subcutaneous immunoglobulin-G (SCIG) may be a reasonable alternative. Severely disabled patients may benefit more from IVIG initially, and SCIG may be considered as a maintenance treatment. The efficacy of SCIG as a maintenance treatment in patients who previously responded to IVIG.
Plasmapheresis is also effective, its efficacy as initial treatment is comparable to IVIG in a 6-week study. Usually, 1 to 1.5 times body volume is exchanged at each session, and 5 to 10 exchanges (2 to 3 exchanges a week as tolerated) may be considered for an initial course of treatment. Plasmapheresis may be used as first-line initial therapy, especially in those with severe symptoms, considering that an improvement is often seen within the first week or after a couple of exchanges. However, more than half of patients have relapsing symptoms within 1 to 2 weeks after stopping plasmapheresis. Its adverse effects may include hemodynamic disturbances, depletion of coagulation factors, and venous access-related complications. Therefore, it may be less ideal as a maintenance therapy option compared with corticosteroids and IVIG.
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For CIDP, there is still a potpourri of unresolved questions including foremost diagnosis and treatment. With multiple advantages in the industry, Creative Biolabs is your reliable partner. If you have any requirements about cell therapy or antibody development, please feel free to contact us for more information.
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