Peter Špalek
Chronic inflammatory demyelinating polyneuropathy (CIDP) belongs to chronic dysimmune (autoimmune) polyneuropathies.
Cardinal clinical criterion is a steadily or stepwise progression of 2 months or more of symmetrical proximal and distal muscle
weakness, with altered sensation and hyporeflexia or areflexia. CIDP is diagnosed according to clinical, electrophysiological,
supportive and exclusive criteria which are a result of a joint task force of the EFNS/PNS in 2010. Diagnosis is founded on clinical
manifestation and course, electrophysiological findings of multifocal demyelinating neuropathy, on some other laboratory findings,
especially proteinocytological dissociation in cerebrospinal fluid, MR findings of hypertrophy and gadolinium-enhanced imaging
of the brachial plexus or spinal nerve, and on clinical improvement on basis of immunotherapy. First choice treatment in
the beginning of therapy is intravenous immunoglobulin (IVIG) or corticosteroids. As CIDP is a treatable disease, early diagnosis
and adequate immunotherapy are decisive for patient´s prognosis. Autoimmune mechanisms cause in CIDP demyelination
of nerve fibres, after longer duration of untreated CIDP an axonal dysfunction develops what is therapeutically difficult to influence.
The severity of prognosis depends on the grade of axonopathy, aptly expressed by the phrase “time is axon”.
In the therapy, when selecting the first-line treatment, it is purposeful to use general EFN/PNS guidelines 2010. The guidelines
lay stress on form and severity of CIDP. IVIG is a treatment of first choice in patients with acute “GBS like” onset, with
severe symptomatology requiring rapid and intensive start of treatment effect, and in pure motor CIDP form in which the
corticotherapy is ineffective. Corticosteroids are treatment of first choice in mild forms of CIDP. In therapy choice it is important
to take into account the patient´s age, comorbidity, an association with other autoimmune diseases, all this requiring
an individualised approach to therapy. CIDP patients show considerable interindividual differences at providing the optimal
therapeutic effects by maintaining treatment (IVIG, Prednisone). The main differences are in the size of maintaining dosage
(IVIG, Prednisone) needed to maintain a long-standing stabile therapeutic effect. A personalised approach with regular monitoring
of patient´s clinical state is decisive for the management of long-maintenance treatment in CIDP. The individualised
approach is especially important in gradual reducing of maintenance doses and in decisions to discontinue the therapy. During
reducing and discontinuing the therapy it is necessary to calculate the risks of CIDP relapses, until now there are no biomarkers
enabling to predict the CIDP relapses.