Neurol. praxi. 2018;19(4):271-275 | DOI: 10.36290/neu.2018.105

Intravenous immunoglobulin in treatment of chronic inflammatory demyelinating polyneuropathy

doc. MUDr. Peter Špalek, PhD.

Centrum pre neuromuskulárne ochorenia, Neurologická klinika SZU a UNB, Nemocnica Ružinov, Bratislava

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 muscleweakness, with altered sensation and hyporeflexia or areflexia. CIDP is diagnosed according to clinical (inclusive, exclusive),electrophysiological and supportive criteria which are a result of a joint task force of the European Federation of NeurologicalSocieties/Peripheral Nerve Society (EFNS/PNS) in 2010. First choice treatment in the beginning of therapy is intravenous immunoglobulin(IVIG) or corticosteroids. In the therapy, when selecting the first-line treatment, it is purposeful to use general EFNS/PNS guidelines. 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 form inwhich the corticotherapy is ineffective. Corticosteroids are treatment of first choice in mild forms of CIDP. In therapy choice it isimportant to take into account the patient´s age, comorbidity, and association with other autoimmune diseases, all this requiringan individualised approach to therapy. CIDP patients show considerable interindividual differences at providing the optimaltherapeutic effects by maintaining treatment (IVIG, Prednisone). The main differences are in the size of maintaining dosage (IVIG, Prednisone) needed to maintain a long-standing stable therapeutic effect. A personalised approach with regular monitoringof patient´s clinical state is decisive for the management of long-maintenance treatment in CIDP. The individualised approachis especially important in gradual reducing of maintenance doses and in decisions to discontinue the therapy. During reducingand discontinuing the therapy it is necessary to calculate the risks of CIDP relapses, until now there are no biomarkers enablingto predict the CIDP relapses. 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 fibers, after longer duration of untreated CIDPan axonal dysfunction develops what is therapeutically difficult to influence. The severity of prognosis depends on the grade ofaxonopathy, aptly expressed by the phrase „time is axon“.

Keywords: CIDP, treatment, IVIG, corticosteroids, general guidelines, personalised approach

Published: September 1, 2018  Show citation