Treatment Of Chronic Inflammatory Demyelinating Polyradiculoneuropathy Research Articles

Updated Diagnosis and Treatment for Chronic Inflammatory Demyelinating Polyradiculoneuropathy Based on the EAN/PNS Guideline 2021

Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) includes a number of clinical subtypes. The major phenotype is "typical CIDP," which is characterized by symmetric and "proximal and distal" muscle weakness. Due to historical changes in the concept of CIDP, multifocal motor neuropathy, anti-myelin-associated glycoprotein (anti-MAG) neuropathy, and autoimmune nodopathy were excluded. International guidelines for the diagnosis and treatment of CIDP were first published in 2005 and revised in 2010. In 2021, the guidelines of the European Academy of Neurology (EAN)/Peripheral Nerve Society (PNS) were the second revision, reflecting changes in the disease concept and progress of electrodiagnosis, neuroimaging, and novel treatments. This review introduces the outline of the guidelines in addition to typical CIDP; related chronic demyelinating neuropathies were classified as CIDP variants. The diagnosis of CIDP is based on (1)the phenotype of a typical CIDP or variant, (2)electrophysiological evidence of peripheral nerve demyelination, and (3)exclusion criteria. The first-line treatments for typical CIDP are corticosteroids and immunoglobulin therapy. These guidelines recommend intravenous or subcutaneous immunoglobulin as maintenance therapy, as well as unresolved questions on the evolving concept of CIDP and future treatments.

Efficacy and safety of combined low-dose rituximab regimen for chronic inflammatory demyelinating polyradiculoneuropathy.

To determine the efficacy and safety of combined low-dose rituximab with conventional therapy for chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) treatment. Total 73 patients with CIDP were enrolled for the retrospective cohort study, and divided into conventional first-line therapy cohort (n = 40) and combined low-dose rituximab (100 mg per infusion) cohort (n = 33). The outcome measures include scores of I-RODS, mRS, INCAT, ONLS, TSS, and COMPASS 31 scale at baseline and regular four visits (4, 16, 28, and 52 weeks), as well as proportion of favorable response and outcome, corticosteroids dosage, and deterioration occurrence during follow-up. Compared to conventional therapy cohort, combined rituximab cohort presented better improvements and higher proportion of favorable response in scales assessments at each visit, as well as significantly reduced corticosteroids dosage and deterioration occurrence during the follow-up. Analyses of subgroups showed better improvements in both typical CIDP and CIDP variants in combined rituximab cohort than those in conventional therapy cohort, but had no differences between each other. Early initiating combined rituximab regimen (<10 weeks) showed better improvements than delayed initiation (≥10 weeks) at the first three visits within 28 weeks, while had no difference in favorable prognoses at the last visit of 52 weeks after once reinfusion. No rituximab correlated serious adverse events were reported in our patients. Our simplified regimen of combined low-dose rituximab has been firstly demonstrated for the better efficacy and safety than conventional therapy in CIDP treatment.

Long-term safety and tolerability of hyaluronidase-facilitated subcutaneous immunoglobulin 10% as maintenance therapy for chronic inflammatory demyelinating polyradiculoneuropathy: Results from the ADVANCE-CIDP 3 trial.

Hyaluronidase-facilitated subcutaneous immunoglobulin (fSCIG) consists of subcutaneous human immunoglobulin G (IgG) 10% with recombinant human hyaluronidase (rHuPH20) and can be administered at the same dose and interval as intravenous IgG (IVIG). fSCIG recently received US approval as maintenance therapy for adults with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) and European approval for adults and children with CIDP after stabilization with IVIG. ADVANCE-CIDP 3 (NCT02955355) was an open-label long-term extension of the Phase 3 double-blind randomized placebo-controlled ADVANCE-CIDP 1 study (NCT02549170) that examined fSCIG safety and efficacy as maintenance CIDP therapy. Primary outcomes were safety, tolerability, and immunogenicity. Efficacy was an exploratory outcome. The study provided 220 patient-years of follow-up data from 85 patients. Median (range) exposure was 33 (0-77) months. Patients received fSCIG every 4 weeks (88.2%) or every 3 weeks (11.8%). Median (range) 4-weekly IgG dose equivalent was 64.0 (28.0-200.0) g. Mean (standard deviation) infusion duration was 135.5 (62.8) minutes. Most adverse events (AEs) were mild or moderate and self-limiting. Of the 1406 AEs, only 48 were severe and 30 were serious. fSCIG-related AEs (n = 798) included infusion site reactions such as pain, redness, and pruritus. Three infusions (0.1%) were reduced in rate, interrupted, or stopped due to intolerability. Relapse occurred in 10 of 77 patients (13.0%); annual relapse rate was 4.5%. An anti-rHuPH20 antibody titer ≥1:160 was detected in 14 of 84 patients (16.7%); patients who tested positive (≥1:160) had similar relapse rates versus those who tested negative (16.7% vs. 12.3%, respectively). ADVANCE-CIDP 3 demonstrated favorable fSCIG long-term safety and tolerability consistent with its established safety profile, and a low relapse rate, supporting use as maintenance CIDP treatment.

Autoimmune nodopathy

AbstractAutoimmune nodopathy (AN) is characterized by the presence of autoantibodies targeting molecules essential for saltatory conduction in myelinated nerves. Clinical manifestations of AN show similarities with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP), including progressive and symmetrical sensorimotor deficits with electrophysiological demyelinating features in nerve conduction studies. Although no common autoantibodies have yet been identified in CIDP, AN is characterized by autoantibodies that primarily target specific molecular complex structures represented by the Ranvier node and paranode. Furthermore, these autoantibodies, such as neurofascin‐155 (NF155), contactin‐1 (CNTN1), contactin‐related protein 1 (Caspr1), and the CNTN1/Caspr1 complex, are illustrative examples of such autoantibodies. They can disrupt the septal‐like junction of paranodes without triggering cellular immune responses. AN manifests uniquely with symptoms such as ataxia, tremors, and markedly high cerebrospinal fluid (CSF) protein levels, often accompanied by nerve root and cranial nerve hypertrophy. Notably, this condition is resistant to immunotherapies typically effective against CIDP, including intravenous immunoglobulin therapy (IVIg). Current evidence suggests that B‐cell depletion therapies, such as rituximab, could benefit AN treatment. Since it has been suggested that existing treatments for CIDP may be effective in cases of autoantibody positivity of subclasses other than IgG4, CIDP that is resistant to conventional therapy requires novel therapeutic strategies that take into account the possibility of IgG4 autoantibodies.

Cutting edge of diagnosis and treatment for chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) based on the EAN/PNS guideline 2021

Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is a most common chronic immune-mediated demyelinating neuropathy, and includes a number of clinical subtypes. The major phenotype is "typical CIDP", which is characterized by symmetric polyneuropathy and "proximal and distal" muscle weakness. During the historical changes in the concept of CIDP, multifocal motor neuropathy, anti-myelin-associated glycoprotein (MAG) neuropathy, and autoimmune nodopathy have been excluded. Currently CIDP is considered as a syndrome including typical CIDP and CIDP variant such as distal CIDP and multifocal CIDP. In 2021, the international guideline of diagnosis and treatment for CIDP, European Academy of Neurology (EAN)/Peripheral Nerve Society (PNS) Guideline, was published. This review article introduces the putline of the guideline with medical-social situation in Japan. The diagnosis of CIDP is based on (1) phenotype of typical CIDP or variant, (2) electrophysiologic evidence of peripheral nerve demyelination, and (3) exclusion criteria. The first-line treatments are corticosteroids or immunoglobulin therapy, and plasma exchange should be considered if the 2 treatments were not effective sufficiently. This guideline recommends intravenous or subcutaneous immunoglobulin as a maintenance therapy, and suggests other immune-suppressive agents. In the near future, new treatment with biologics, such as monoclonal antibodies against neonatal Fc receptors, complements, and CD19/20 will be approved.

Chronic Inflammatory Demyelinating Polyradiculoneuropathy Misdiagnosed as Amyotrophic Lateral Sclerosis in a Patient with Diabetes Mellitus

Gradual progressive weakness in a patient with diabetes mellitus can be caused by several conditions, including myelopathy, motor neuron disease, chronic inflammatory demyelinating polyradiculoneuropathy (CIDP), and myopathy. Diagnosing the exact cause of progressive muscle weakness can be challenging in the presence of diabetic polyneuropathy (DPN). We report a case of CIDP with DPN in a 64-year-old woman who was initially misdiagnosed with amyotrophic lateral sclerosis. This case also demonstrates the importance of using the correct dose and duration for the treatment of CIDP.

Chronic inflammatory demyelinating polyradiculoneuropathy concomitant with nephropathy.

Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is one of the most common autoimmune peripheral neuropathies in adults. Membranous nephropathy (MN), focal segmental glomerulosclerosis (FSGS), and other nephropathy have been reported in CIDP patients and are possibly correlated to CIDP pathogenesis. This study reviewed the previously described cases of patients with CIDP and nephropathy in order to provide comprehensive evidence on the diagnosis and treatment regarding CIDP patients in the context of renal diseases. We reviewed our database to identify patients with CIDP and nephropathy. Online database including PubMed, EMBASE, and OVID were searched for relevant cases. We identified a total of 18 cases with CIDP and nephropathy, including 2 cases from our database and 16 ones from online searching. A predominance of male was observed [14 (77.8%)] with the mean age of 53.3 (standard deviation, SD: 16.6) years old. Almost all patients complained paresthesia in distal limbs (94.4%), except one only presented weakness of four extremities. Corticosteroids were prescribed for 14 (77.8%) patients, and 10 showed responsiveness. Three patients experienced relapses during the gradual tapering of steroids. The same immune-mediated pathogenesis may be involved in CIDP and concomitant nephropathy. Male and sensory-predominant CIDP are red flags for complications of renal diseases in CIDP patients. Corticosteroids remain the first-line treatment for CIDP when complicated with renal diseases. Slower tapering or long-term maintenance of steroids may be beneficial for the prognosis of patients with CIDP and nephropathy.

Treatment Options for Chronic Inflammatory Demyelinating Polyradiculoneuropathy (CIDP): With a Focus on Immunoglobulin Treatment

The disease state and clinical course of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) are diverse, and the response to treatment varies from patient to patient. Therefore, it is necessary to select a treatment and determine the appropriate dose and dosage interval while monitoring the clinical course after treatment initiation. In this article, we will discuss points to be considered and the results of studies that can be used as references in order to make an appropriate choice at each stage of CIDP treatment.

A Review of the History of CIDP Treatment: From Symptom Improvement to Maintenance Therapy- with a Focus on SCIg

Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is an autoimmune peripheral neuropathy with chronic progression over 2 months or more. In this review, we provide an overview of key clinical studies involved in the development of CIDP therapy, as well as a discussion of changes in the concept of treatment. Although a definitive therapy has not yet been established, international and Japanese clinical guidelines recommend three first-line treatment options for symptom improvement, namely corticosteroids, intravenous immunoglobulin (IVIg), and plasmapheresis, based on the results of clinical studies conducted before the early 2000s. Since 2010, several treatments for the prevention of CIDP relapse (maintenance therapy) have been developed and more recently, studies have focused on the optimization of each treatment. On the other hand, CIDP treatment is associated with several limitations, including a lack of biomarkers for prediction of disease progression, differences in response to treatment between CIDP subtypes, and difficulties in the selection of appropriate maintenance therapy. Several studies aimed at resolving these issues are currently being conducted. (Received 22 May, 2020; Accepted 12 January, 2021; Published 1 July, 2021).

Pain in Chronic Inflammatory Demyelinating Polyradiculoneuropathy: A Systematic Review and Meta-Analysis.

IntroductionAlthough chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) predominantly affects large myelinated fibers, many patients have pain. The aim of this paper is to systematically review the current literature regarding CIDP with a particular focus on epidemiological and clinical characteristics of painful CIDP.MethodsA systematic literature search was conducted on PubMed database.ResultsOur search strategy identified 146 articles. Thirty-eight papers, reporting on 991 patients, met the inclusion criteria and were used for this review. The pooled estimate of the prevalence of pain at any point within the course of CIDP was 46% (95% CI 36–57%). Immune treatment of CIDP might be adequate as monotherapy for the management of pain. Treatment specific to pain currently shows effectiveness as adjuvant treatment when CIDP is treated and pain persists.ConclusionsPain in CIDP is an underexplored field. Future research should focus on the natural history, phenomenology, and management of pain in CIDP.

Oral fingolimod for chronic inflammatory demyelinating polyradiculoneuropathy (FORCIDP Trial): a double-blind, multicentre, randomised controlled trial

Fingolimod is approved for the treatment of relapsing-remitting multiple sclerosis and was effective in experimental autoimmune neuritis in rats, a possible model for chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). We aimed to evaluate the efficacy of fingolimod in delaying disability progression in patients with CIDP who withdrew from currently effective treatments (intravenous immunoglobulin [IVIg] or corticosteroids). This double-blind, multicentre, randomised, placebo-controlled, parallel-group, event-driven study was done at 48 neurology centres in Australia, Canada, Israel, Japan, the USA, and nine countries in Europe. Participants with CIDP who were receiving IVIg or corticosteroids were randomly assigned (1:1) to once-daily oral fingolimod 0·5 mg or placebo. Owing to the event-driven design, treatment duration was flexible and could be up to 4·5 years. Randomisation was done with an automated interactive voice response-web response system and was stratified by Inflammatory Neuropathy Cause and Treatment (INCAT) disability scale scores. Previous IVIg treatment was discontinued after one final course ending the day before the first dose of fingolimod or placebo was given, whereas corticosteroids were tapered off over 8 weeks after randomisation. The primary endpoint was time to first confirmed worsening (≥1 point increase on the adjusted INCAT disability scale score versus baseline) and was assessed in the full analysis set, which consisted of all patients who underwent randomisation and had at least one efficacy assessment for the primary analysis. The survival distribution functions of time to first worsening were estimated within each treatment group according to the Kaplan-Meier survival distribution function and compared with a stratified log-rank test. The trial is registered with ClinicalTrials.gov, number NCT01625182. Of 106 participants randomly assigned between Jan 24, 2013, and March 10, 2016, 54 received fingolimod (41 who had been receiving IVIg and 13 who had been receiving corticosteroids) and 52 received placebo (41 who had been receiving IVIg and 11 who had been receiving corticosteroids). The trial ended for futility as recommended by an independent data monitoring committee after an interim analysis when 44 confirmed worsening events had occurred. At the end of the study, the survival estimate of the proportion of participants free from confirmed worsening was not significantly different between the fingolimod group (42%, 95% CI 23-60) and the placebo group (43%, 28-59; p=0·91). Adverse events occurred in 41 (76%) participants receiving fingolimod and 44 (85%) on placebo, and serious adverse events occurred in nine (17%) and four (8%) patients, respectively. The most common adverse events with fingolimod were headache (12 [22%] patients), hypertension (ten [19%]), and extremity pain (seven [13%]). Adverse events leading to study discontinuation occurred in seven (13%) participants on fingolimod and none on placebo. Fingolimod 0·5 mg once-daily was not better than placebo for the treatment of CIDP. Future trial designs should take account of the possibility that if IVIg is stopped abruptly, some patients might relapse soon afterwards whereas others might remain in remission. Novartis Pharma.

Corticosteroids for chronic inflammatory demyelinating polyradiculoneuropathy.

Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is a progressive or relapsing and remitting paralysing illness, probably due to an autoimmune response, which should benefit from corticosteroid treatment. Non-randomised studies suggest that corticosteroids are beneficial. Two commonly used corticosteroids are prednisone and prednisolone. Both are usually given as oral tablets. Prednisone is converted into prednisolone in the liver so that the effect of the two drugs is usually the same. Another corticosteroid, dexamethasone, is more potent and is used in smaller doses. The review was first published in 2001 and last updated in 2015; we undertook this update to identify any new evidence. To assess the effects of corticosteroid treatment for CIDP compared to placebo or no treatment, and to compare the effects of different corticosteroid regimens. On 8 November 2016, we searched the Cochrane Neuromuscular Specialised Register, Cochrane Central Register of Controlled Trials, MEDLINE, and Embase for randomised trials of corticosteroids for CIDP. We searched clinical trials registries for ongoing trials. We included randomised controlled trials (RCTs) or quasi-RCTs of treatment with any corticosteroid or adrenocorticotrophic hormone for CIDP, diagnosed by an internationally accepted definition. Two authors extracted data from included studies and assessed the risk of bias independently. The intended primary outcome was change in disability, with change in impairment after 12 weeks and side effects as secondary outcomes. We assessed strength of evidence using the GRADE approach. One non-blinded RCT comparing prednisone with no treatment in 35 eligible participants did not measure the primary outcome for this systematic review. The trial had a high risk of bias. Neuropathy Impairment Scale scores after 12 weeks improved in 12 of 19 participants randomised to prednisone, compared with five of 16 participants randomised to no treatment (risk ratio (RR) for improvement 2.02 (95% confidence interval (CI) 0.90 to 4.52; very low-quality evidence). The trial did not report side effects in detail, but one prednisone-treated participant died.A double-blind RCT comparing daily standard-dose oral prednisolone with monthly high-dose oral dexamethasone in 40 participants reported none of the prespecified outcomes for this review. The trial had a low risk of bias, but the quality of evidence was limited as it came from a single small study. There was little or no difference in number of participants who achieved remission (RR 1.11; 95% CI 0.50 to 2.45 in favour of monthly dexamethasone; moderate-quality evidence), or change in disability or impairment after one year (low-quality evidence). Change of grip strength or Medical Research Council (MRC) scores demonstrated little or no difference between groups (moderate-quality to low-quality evidence). Eight of 16 people in the prednisolone group and seven of 24 people in the dexamethasone group deteriorated. Side effects were similar with each regimen, except that sleeplessness was less common with monthly dexamethasone (low-quality evidence) as was moon facies (moon-shaped appearance of the face) (moderate-quality evidence).Experience from large non-randomised studies suggests that corticosteroids are beneficial, but long-term use causes serious side effects. We are very uncertain about the effects of oral prednisone compared with no treatment, because the quality of evidence from the only RCT that exists is very low. Nevertheless, corticosteroids are commonly used in practice, supported by very low-quality evidence from observational studies. We also know from observational studies that corticosteroids carry the long-term risk of serious side effects. The efficacy of high-dose monthly oral dexamethasone is probably little different from that of daily standard-dose oral prednisolone. Most side effects occurred with similar frequencies in both groups, but with high-dose monthly oral dexamethasone moon facies is probably less common and sleeplessness may be less common than with oral prednisolone. We need further research to identify factors that predict response.

Maintenance IV immunoglobulin treatment in chronic inflammatory demyelinating polyradiculoneuropathy.

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.

Immunomodulatory treatment other than corticosteroids, immunoglobulin and plasma exchange for chronic inflammatory demyelinating polyradiculoneuropathy.

Immunomodulatory treatment other than corticosteroids, immunoglobulin and plasma exchange for chronic inflammatory demyelinating polyradiculoneuropathy.

Treatments for chronic inflammatory demyelinating polyradiculoneuropathy (CIDP): an overview of systematic reviews.

Treatments for chronic inflammatory demyelinating polyradiculoneuropathy (CIDP): an overview of systematic reviews.

Chronic Inflammatory Demyelinating Polyradiculoneuropathy 101—Pitfalls and Pearls of Diagnosis and Treatment

Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP), which is caused by demyelination of the peripheral nerves, is characterized by progressive weakness and impaired sensory function in the arms and legs. CIDP is a treatable condition in which early diagnosis is crucial to limit chronic disability. CIDP can mimic other neuropathies and it is important to identify these in order to ensure prompt treatment. Patients with other causes of neuropathy should be suspected of having CIDP if there is rapid progress or proximal weakness. Intravenous immunoglobulin (IVIG), corticosteroids, and plasma exchange are first-line therapies. The IVIG CIDP Efficacy (ICE) trial, the largest trial reported of any CIDP treatment, demonstrated that IVIG therapy reduced disability and functional impairment, as well as improved quality of life. Autoantibodies against membrane proteins of the peripheral nerve axons or the myelin sheath have been reported recently, and an improved understanding of antibody responses in CIDP may enable the development of future targeted therapeutic interventions.

Intravenous immunoglobulin treatment in chronic inflammatory demyelinating polyradiculoneuropathy, a time to start and a time to stop.

Intravenous immunoglobulin (IVIg) is often used as preferred treatment in chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). Several studies highlighted the short-term efficacy of IVIg for CIDP yet many patients need maintenance therapy. Notwithstanding the fact IVIg has been used for over 30 years in CIDP, there is only limited evidence to guide dosage and interval during maintenance treatment. The variation in disease course, lack of biomarkers, and fear of deterioration after stopping IVIg makes long-term treatment challenging. Recent studies suggest a proportion of patients receive unnecessary IVIg maintenance treatment. This review provides an overview of the use of IVIg for CIDP treatment, focusing on evidence for long-term IVIg use.

Intravenous methylprednisolone versus intravenous immunoglobulin in chronic inflammatory demyelinating polyradiculoneuropathy: a randomized trial

Introduction: Corticosteroids and Intravenous immunoglobulin (IVIg) are effective as initial treatment in patients with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP), but their long-term use is less defined. We compared the efficacy and tolerability of 6-month therapy of intravenous methylprednisolone (IV MP) versus IVIg. Methods: This trial compared efficacy and tolerability of IVIg (0·5g/kg per day for 4 consecutive days) and IV MP (0·5 g in 250 ml sodium chloride solution per day for 4 consecutive days) given every month for 6 months were assessed. After therapy discontinuation, patients were followed up for 6 months to assess relapses. Results: 40 patients (20 IV MP, 20 IVIg) completed the study. More patients stopped MP (12 [60%] of 20) than IVIg (8 [40%] of 20; relative risk 0·54, 95% CI 0·34–0·87; p=0·0085). When adjusted for sex, age, disease duration, comorbidity, modified Rankin scale (mRS) at enrolment, and previous treatment with IVIg and steroids, the difference between the two groups remained significant (odds ratio 7·6, 95% CI 1·7–33·8; p=0·0070). Reasons for discontinuation were lack of efficacy (twelve in the IV MP group vs. 8 in the IVIg group), adverse events (one in the IV MP group), or voluntary withdrawal (2 in IV MP group). The proportion of patients with adverse events did not differ between IV MP group (14 [67%] of 20) and IVIg group (11 [46%] of 20; p=0·1606). After discontinuation, more patients on IVIg worsened and required further therapy (eight [38%] of 20, p=0·0316). Conclusion: Treatment of CIDP with IVIg for 6 months was less frequently discontinued than with IV MP.

Novel immunotherapeutic strategies in chronic inflammatory demyelinating polyneuropathy.

Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is a chronic immune-mediated neuropathy: it is clinically heterogeneous (relapsing-remitting form, chronic progressive form, monophasic form or CIDP having a Guillain-Barré syndrome-like onset), but potentially treatable. Although its pathophysiology remains largely unknown, CIDP is considered an immune-mediated neuropathy. Therefore, many immunotherapies have been proposed in this peripheral nervous system disorder, the most known efficient treatments being intravenous immunoglobulin, corticosteroids and plasma exchange. However, these therapies remain unsatisfactory for many patients, so numerous other immunotherapeutic strategies have been evaluated, based on their immunosuppressant or immunomodulatory potency. We have performed a large review of the literature about treatment in CIDP, with a special emphasis on novel and alternative immunotherapeutic strategies.

New insights into the management of chronic inflammatory demyelinating polyradiculoneuropathy.

Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) and its variants can be challenging to diagnose and treat. A combination of clinical, electrophysiological and laboratory features is often required to reach a diagnosis. New data are emerging about potential biomarkers and factors that may indicate treatment needs in individual patients. High-quality evidence exists for the efficacy of intravenous immunoglobulin (IVIG) in the treatment of CIDP, including quality of life (QoL) benefits. Besides pharmacological treatment, psychological factors must also be addressed to improve patients' QoL. Home-based IVIG infusion therapy is currently a well-established approach in some countries. A 6-month pilot study conducted in Ontario, Canada, provided proof of safety and patient acceptance of home-based IVIG therapy, although some logistical issues emerged.