2019 Volume 36 Issue 4 Pages 439-444
Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is an acquired peripheral neuropathy characterized by demyelination triggered by autoimmune–responses that targets the myelin sheath or paranodal molecules. Clinically, it has monophasic, relapsing, not only slowly progressive course, and causes motor sensory dysfunction in each limb. The disease is characterized by its diversity in the distribution of disorders and therapeutic responses, etc. Although the pathogenesis of both cellular and humoral immunity is presumed to be involved, it is not a uniform immunological background, but diverse. It is considered as a syndrome in which various pathological conditions co–exist. Therefore, understanding of the diversity and understanding of the characteristics of each subtype are essential from the viewpoint of therapeutic intervention as well as elucidation of the pathological condition.
According to the conventional criteria (EFNS/PNS criterion), CIDP is broadly classified into typical CIDP and non–typical CIDP, and the former has good responsiveness to existing first–line therapeutics. On the other hand, atypical CIDPs are important for differentiation from other diseases, and generally include many intractable cases showing resistance to first–line therapeutics. treatment, there are also subtypes whose pathogenesis is becoming clear. Since IgG4 itself is considered to be essential factor for IgG4–subclass autoantibody positive CIDP, its removal can be expected as a therapeutic. From this, the efficacy of rituximab, which selectively injures B cells, is underway as a doctor–initiated trial in Japan. In addition, even if treatment response is good in the typical clinical picture of CIDP, treatment–dependent cases that repeat recurrence may shift to irreversible axonal degeneration if the proper therapeutic intervention is missed, which may be a prognostic case. Thus, for cases in which frequent relapses occur and where a single initial treatment alone is considered inadequate, the introduction of maintenance therapy should be considered.
