Abstracts of the 8th Meeting of the Italian Peripheral Nerve Study Group: 44

Abstract

50–60% of CIDP patients show a clinical remission with the administration of standard therapies. Unfortunately, the achievement of this goal does not conclude patients’ troubles but coincide with the beginning of another story whose course is often uncertain. In our study of CIDP patients we attempted to establish the fate of 36 patients showing an initial remission after treatments. Over a long‐term period of follow‐up, we observed that the disease in these patients can follow 3 different clinical courses. A first group of 11 patients had a monophasic disease. All of them achieved a complete remission after therapy with corticosteroids and no relapses were observed after drug interruption over a follow‐up period of 6 months‐12 years. The duration of treatment varied between 3–24 months. A second group of 6 patients had a relapsing course; these patients became asymptomatic with corticosteroids but relapsed after a period of time of at least six months (range 6 months‐16 years) since therapy suspension. The number of relapses varied from 1 to 4. A third group was represented by patients (19 in our series) who needed continuous treatment over a long period of time to maintain improvement. The apparent relapsing‐remitting course in these patients was always related to tapering of therapies. The duration of this chronic active form ranged from 2 to 17 years. 5 patients of the last group were able to top therapies without evidence of worsening over a follow‐up period of 2–12 years. This subgroup of patients had a prolonged monophasic course rather than a relapsing‐remitting form. Our findings show that CIDP is a heterogeneous disease encompassing cases with a single monophasic episode in which the term “chronic”, with respect to GBS, is referred mainly to the prolonged phase of worsening and cases in which the chronic nature of the condition in outlined by the long duration (up to decades) of the “active phase”, made‐up of therapy‐related relapses/remissions.