Abstract
ObjectiveTo assess clinical outcome in treatment-naive patients with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP).MethodsWe included adult treatment-naive patients participating in the prospective International CIDP Outcome Study (ICOS) that fulfilled the European Federation of Neurological Societies/Peripheral Nerve Society (EFNS/PNS) diagnostic criteria for CIDP. Patients were grouped based on initial treatment with (1) intravenous immunoglobulin (IVIg), (2) corticosteroid monotherapy or (3) IVIg and corticosteroids (combination treatment). Outcome measures included the inflammatory Rasch-built overall disability scale (I-RODS), grip strength, and Medical Research Council (MRC) sum score. Treatment response, treatment status, remissions (improved and untreated), treatment changes, and residual symptoms or deficits were assessed at 1 year.ResultsForty patients were included of whom 18 (45%) initially received IVIg, 6 (15%) corticosteroids, and 16 (40%) combination treatment. Improvement on ≥ 1 of the outcome measures was seen in 31 (78%) patients. At 1 year, 19 (48%) patients were still treated and fourteen (36%) patients were in remission. Improvement was seen most frequently in patients started on IVIg (94%) and remission in those started on combination treatment (44%). Differences between groups did not reach statistical significance. Residual symptoms or deficits ranged from 25% for neuropathic pain to 96% for any sensory deficit.ConclusionsImprovement was seen in most patients. One year after the start of treatment, more than half of the patients were untreated and around one-third in remission. Residual symptoms and deficits were common regardless of treatment.
Highlights
Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is a rare and heterogeneous immune-mediated neuropathy, usually causing progressive weakness of the extremities with sensory dysfunction
Eleven patients were excluded due to an alternative diagnosis, and a further 202 excluded from the present study for the following reasons: not treatment-naive (n = 165), follow-up < 1 year (n = 21), participation in the OPTIC trial (n = 8), never treated (n = 6) or not fulfilling the electrodiagnostic criteria for CIDP (n = 2), resulting in 40 patients included for analysis
Patients treated with Intravenous immunoglobulins (IVIg) were classified as ‘typical’ CIDP more often compared to patients treated with corticosteroids or both, this was not significantly different
Summary
Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is a rare and heterogeneous immune-mediated neuropathy, usually causing progressive weakness of the extremities with sensory dysfunction. Intravenous immunoglobulins (IVIg), corticosteroids, and plasma exchange (PE) are considered effective first-line treatments for CIDP [6,7,8,9]. Most neurologists use either IVIg or corticosteroids as initial treatment [8, 10, 11]. Both treatments have their own distinct advantages and disadvantages. IVIg often leads to rapid improvement in disability and has a low risk of serious adverse events, but the benefit is usually short-lived requiring long-term repeated infusions which carries a significant economic burden [9, 12,13,14]. Using data from the International CIDP Outcome Study (ICOS), a prospective observational cohort in three Dutch tertiary neuromuscular centers, we aimed to assess response to treatment, treatment status, remission, and residual symptoms or deficits in treatment-naive patients with CIDP 1 year after the start of treatment
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