Abstract:

Chronic inflammatory demyelinating polyneuropathy (CIDP) is a treatable immune-mediated disorder, which causes in its typical form, symmetric proximal and distal weakness with large fibre sensory impairment involving the four limbs. There are currently three main first-line therapeutic options for CIDP. These consist of corticosteroids, immunoglobulins and plasma exchanges (PE) which have all been found effective in a number of trials conducted over the past several years. No immunosuppressant therapy has shown benefit in CIDP, although they are utilized by many clinicians in various circumstances despite absence of an evidence base. CIDP is a heterogeneous entity and also consists of so-called “atypical forms”. These can be anatomical with focal and multifocal subtypes, or relate to the nerve fibre type involved, with pure sensory and pure motor variants. There are also forms co-existing with associated diseases. There are likely different pathophysiologic mechanisms for the different subtypes which may in turn affect best treatment to be offered for each variant. An example is the pure motor form of CIDP, for which there are a number of reports which have described deterioration on steroids, making immunoglobulins the favoured first-line treatment. The degree of electrophysiological, albeit asymptomatic, sensory involvement may hence also represent a marker of corticosteroid-responsiveness, as may also the degree of focal electrophysiological demyelination. Co-existing disease, such as diabetes may make use of certain treatments such as corticosteroids unadvisable.
In conclusion, therapeutic decision-making in CIDP requires consideration of a number of different factors, relating principally to the individual patient’s circumstances. Disease severity, disease sub-type, age, comorbidities, all play a significant role in the process. First-line therapies most frequently suffice alone and in combination, and effects should be carefully evaluated using appropriate validated scales. Immunosuppressant treatment, although without an evidence base, should not be excluded in selected, albeit exceptional cases. The task of tailoring CIDP therapy for each patient is important, with often long-term implications. The right decision may not be easy but is crucial both in order to offer the maximum chances of remission and/or cure, while offering the justifiably most adequate therapeutic option for every affected individual in relation to risk exposure and side-effect profile.