Treatment dependence in chronic inflammatory demyelinating polyradiculoneuropathy: Is it related to disease phenotype or to the therapy used?

摘要

Chronic inflammatory demyelinating poly-radiculoneuropathy (CIDP) is a rare and disabling immune neuropathy which often has a heterogeneous presentation. Patients usually improve after therapy with steroids, high-dose intravenous immunoglobulin (IVIg) or plasma exchange. Most patients require, however, a prolonged treatment to avoid deterioration. It is unclear which therapy should be first used in these patients and whether there are clues to predict for how long therapy should be continued. Rabin et al reviewed the data from 70 patients with CIDP who had responded to therapy and compared the clinical, electrophysiological and laboratory data of 36 patients who could suspend therapy without deterioration (treatment withdrawal) with those of 34 patients who needed to continue the therapy to avoid deterioration (treatment dependent).