Maintenance IV immunoglobulin treatment in chronic inflammatory demyelinating polyradiculoneuropathy

Abstract

Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) patients treated with intravenous immunoglobulin (IVIg) usually start with a standard dosage of 2 g/kg bodyweight. Only a minority of patients has a sustained improvement, and most require ongoing maintenance treatment. Preferred IVIg regimens, however, vary considerably between doctors and at present it is unknown which is optimal. As there are also large differences in IVIg dosage and interval requirements between patients, optimal IVIg maintenance treatment of CIDP is even more complex. The lack of evidence-based guidelines on how IVIg maintenance treatment should be administered may potentially lead to under- or overtreatment of this expensive therapy. We provide an overview of published practical IVIg maintenance treatment regimens, IVIg maintenance schedules used in randomized controlled trials and one based upon our own long-term experience on how this treatment could be given in CIDP.

Clinical factors, diagnostic delay, and residual deficits in chronic inflammatory demyelinating polyradiculoneuropathy

“…When patients receive maintenance treatment, stepwise tapering of the dose or extension of the interval is essential to determine disease activity in patients and prevent overtreatment. Algorithms to reduce IVIg doses according to the patients' individual needs are suggested …”

“…The treatment of CIDP is complicated by the variable responsiveness of individual patients. Although dosing algorithms for IVIg treatment are suggested, there is no gold standard available on how to select treatment type, regimen, and how to monitor maintenance treatments . Early diagnosis and optimal treatment are important to prevent secondary axonal nerve damage which can cause permanent disability in patients .…”

“…Although dosing algorithms for IVIg treatment are suggested, there is no gold standard available on how to select treatment type, regimen, and how to monitor maintenance treatments. 20,21 Early diagnosis and optimal treatment are important to prevent secondary axonal nerve damage which can cause permanent disability in patients. 22,23 Treatment responsiveness varies among patients, this may lead to under treatment, as well as overtreatment when the disease is in remission, as shown in two randomized controlled studies where 16% to 55% of placebo treated CIDP patients did not relapse after discontinuation of treatment.…”

Diagnosis and treatment of chronic inflammatory demyelinating polyradiculoneuropathy in clinical practice: A survey among Dutch neurologists

“…15 Furthermore, the best approach to manage wear-off signs and withdrawal of IVIg is unclear. 16,17 Because of these challenges, we expect that both the diagnostic workup and treatment strategies for CIDP patients are highly variable.…”

Individualized immunoglobulin therapy in chronic immune‐mediated peripheral neuropathies*

“…In a cohort of 71 patients (including 39 with CIDP and 24 with MMN) utilization of this protocol resulted in a mean (± standard error) dose of 1.4 ± 0.6 g/kg at a mean interval of 4.3 weeks (range 0.5 to 10 weeks) (Lunn et al, ) . A similar, but not identical, approach has been proposed by the Department of Neurology Erasmus MC (Kuitwaard et al, ) . Neither the Lunn nor the Erasmus MC approaches were designed to be dogmatic, but both highlight important principles that should be considered in any optimization approach.…”