Chronic Inflammatory Demyelinating Polyradiculoneuropathy (CIDP)

A slowly progressive autoimmune demyelinating disease of peripheral nerves and nerve roots. Clinical manifestations include weakness and sensory loss in the extremities and enlargement of peripheral nerves. The course may be relapsing-remitting or demonstrate a step-wise progression. Protein is usually elevated in the spinal fluid and cranial nerves are typically spared. GUILLAIN-BARRE SYNDROME features a relatively rapid progression of disease which distinguishes it from this condition. (Adams et al., Principles of Neurology, 6th ed, p1337)

Also Known As:

CIDP; Polyradiculoneuropathy, Chronic Inflammatory; Polyradiculoneuropathy, Chronic Inflammatory Demyelinating; Chronic Inflammatory Polyradiculoneuropathy; Inflammatory Polyradiculopathy, Chronic; Polyneuropathy, Inflammatory Demyelinating, Chronic; Chronic Inflammatory Polyradiculoneuropathies; Chronic Inflammatory Polyradiculopathies; Chronic Inflammatory Polyradiculopathy; Inflammatory Polyradiculoneuropathies, Chronic; Inflammatory Polyradiculoneuropathy, Chronic; Inflammatory Polyradiculopathies, Chronic; Polyradiculoneuropathies, Chronic Inflammatory; Polyradiculopathies, Chronic Inflammatory; Polyradiculopathy, Chronic Inflammatory

Networked: 1396 relevant articles (257 outcomes, 301 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Chronic Inflammatory Demyelinating Polyradiculoneuropathy (CIDP)
2.	Polyneuropathies (Polyneuropathy)
3.	Guillain-Barre Syndrome
4.	Myasthenia Gravis
5.	Dermatomyositis (Dermatopolymyositis)

Experts

1.	Hartung, Hans-Peter: 39 articles (01/2022 - 04/2002)
2.	Nobile-Orazio, Eduardo: 32 articles (06/2022 - 11/2003)
3.	Bril, Vera: 30 articles (01/2022 - 02/2008)
4.	van Schaik, Ivo N: 27 articles (01/2022 - 12/2002)
5.	Rajabally, Yusuf A: 27 articles (12/2021 - 06/2004)
6.	Dalakas, Marinos C: 26 articles (01/2022 - 12/2002)
7.	van Doorn, Pieter A: 26 articles (01/2022 - 06/2005)
8.	Querol, Luis: 24 articles (06/2022 - 03/2013)
9.	Merkies, Ingemar S J: 24 articles (01/2022 - 02/2008)
10.	Eftimov, Filip: 23 articles (01/2022 - 01/2009)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Chronic Inflammatory Demyelinating Polyradiculoneuropathy:

1.	Intravenous Immunoglobulins (IVIG)FDA Link 12/15/2020 - "Efficacy of IVIg for CIDP was proven in a large phase III trial. " 11/01/2010 - "Confirming the efficacy of intravenous immunoglobulin in CIDP through minimum clinically important differences: shifting from statistical significance to clinical relevance." 03/01/2018 - "The main objective is to investigate whether high frequent low dosage IVIg treatment is more effective than low frequent high dosage IVIg treatment as maintenance treatment for CIDP. " 01/01/2017 - "The efficacy of SC-Ig is similar to IVIg for CIDP and MMN and has a significant safety profile. " 01/01/2015 - "High-quality evidence exists for the efficacy of intravenous immunoglobulin (IVIG) in the treatment of CIDP, including quality of life (QoL) benefits. "
2.	Adrenal Cortex Hormones (Corticosteroids)IBA 05/01/2009 - "Corticosteroids have been beneficial in controlled trials for CIDP. " 03/26/2002 - "Patients with apparent GBS who show persistent symptoms may benefit from corticosteroids or other treatment that is beneficial in the management of CIDP." 10/01/2022 - "Corticosteroids remain the first-line treatment for CIDP when complicated with renal diseases. " 08/15/2012 - "To evaluate the efficacy of corticosteroid treatment compared to placebo or no treatment for CIDP and to compare the efficacy of different corticosteroid regimes. " 01/01/2002 - "To evaluate the efficacy of corticosteroids for treating chronic inflammatory demyelinating polyradiculoneuropathy. "
3.	Immunoglobulins (Immunoglobulin)IBA 01/01/2019 - "Subcutaneous immunoglobulins (SCIgs) have proven to be effective in adults with CIDP and in children affected by antibody deficiencies and other different immune and inflammatory disorders. " 11/01/2022 - "More robust, reliable, and relevant outcome measures are needed to detect early improvement in immunoglobulin-treated CIDP." 11/01/2014 - "A sustained clinical efficacy was observed after the switch to subcutaneous immunoglobulin: the Overall Neuropathy Limitation Scale score improved in the group of 66 CIDP patients (P = 0.018), with only one subject reporting a worsening of 1 point, and remained stable in the group of 21 MMN patients (P = 0.841), with one subject reporting a worsening of two points. " 01/01/2021 - "The subcutaneous immunoglobulin (SCIg) administration route is a safe and effective alternative option, approved by the United States Food and Drug Administration (FDA) in 2018, for maintenance treatment of adults with CIDP. " 01/01/2015 - "Switch from intravenous to subcutaneous immunoglobulin in CIDP and MMN: improved tolerability and patient satisfaction."
4.	SteroidsIBA 09/12/2017 - "Most patients with CIDP are treated successfully with steroids if the diagnosis is made early. " 10/01/2022 - "Slower tapering or long-term maintenance of steroids may be beneficial for the prognosis of patients with CIDP and nephropathy." 01/01/1994 - "These differences may explain the empirical observations that steroids are helpful in CIDP but not GBS. " 05/01/2020 - "[Variables associated with a good response to steroids in chronic inflammatory demyelinating polyradiculoneuropathy]." 05/01/2019 - "The use of steroids alone was the most common prescribed treatment for CIDP. "
5.	Rituximab (Mabthera)FDA Link 12/29/2020 - "We report a woman with severe refractory CIDP and coexisting chronic lymphocytic leukaemia (CLL) who improved dramatically after chemoimmunotherapy appropriate for the CLL, including rituximab. " 08/24/2004 - "Rituximab may represent a successful therapy in otherwise refractory CIDP." 04/01/2020 - "The results of the RECIPE trial are expected to provide evidence for the positioning of rituximab as a pathogenesis-based therapeutic for refractory CIDP. " 03/01/2022 - "Rituximab was effective in the treatment in CIDP patients, especially in anti-IgG4 antibody-positive patients. " 12/01/2021 - "Rituximab may be effective in refractory CIDP, even though worsening may occur in some patients. "
6.	AntibodiesIBA 01/01/2022 - "A total of 173 patients who underwent HSCT were found, including 32 patients described in case reports and 60 in a phase 2 clinical trial with CIDP, 29 patients with SPS, 42 patients with NMOSD and 10 patients with refractory MG. Complete remission was documented in 68/92 patients with CIDP, 13/29 with SPS and 10/10 with MG. From the NMOSD cases, 24/42 were relapse-free at last follow-up, with 13/33 having negative anti-AQ4 antibodies after HSCT. " 06/01/2005 - "The frequency of such antibodies was significantly greater in both CIDP (p = 0.04) and GBS (p = 0.003) patients than in normal control subjects. " 12/01/2020 - "We present a case of refractory CIDP effectively treated with OCR, switched from RTX after the development of anti-drug antibodies. " 09/01/1995 - "It was concluded that these antibodies may help to distinguish CIDP from other neuropathies and that they are diagnostically useful. " 01/01/2022 - "Although the pathogenesis of CIDP remains to be elucidated, the role of antibodies in the pathogenesis of TBIRS and CIDP might be a promising platform for further studies to provide additional insights into the origin of these 2 rare complications."
7.	Prednisone (Sone)FDA LinkGeneric 03/01/2000 - "The occurrence of the oedema in a person with CIDP and FSGN and its improvement with prednisone, together with improvement in CIDP and FSGN, suggests that it was immune mediated, possibly due to increased capillary permeability. " 04/01/2010 - "Since the first clinical trial of prednisone in CIDP, several randomized trials have given rise to evidence-based therapeutic approaches. " 01/01/1978 - "Suggestive preliminary evidence from controlled 3-month clinical trials that prednisone improves chronic inflammatory polyradiculoneuropathy." 10/16/2021 - "CIDP patients treated with prednisone have good long-term functional clinical response. " 07/01/2009 - "The curative effect of GTC combined with prednisone in treating CIDP was better than that of prednisone alone."
8.	Cyclophosphamide (Cytoxan)FDA LinkGeneric 01/01/2022 - "Cyclophosphamide for Treatment of Refractory Chronic Inflammatory Demyelinating Polyradiculoneuropathy: A Systematic Review and Meta-analysis." 10/01/2007 - "Three MS and five CIDP patients received cyclophosphamide (200 mg/kg) for refractory disease. " 10/01/2007 - "High dose cyclophosphamide preferentially targets naïve T (CD45/CD4/RA+) cells in CIDP and MS patients." 03/01/2005 - "High-dose cyclophosphamide can markedly improve functionality and quality of life for patients with severe refractory CIDP." 06/25/2002 - "High-dose cyclophosphamide without stem-cell rescue for refractory CIDP."
9.	Proteins (Proteins, Gene)FDA Link 01/01/2023 - "Confirmed CIDP patients usually had their initial diagnosis based on the nerve conduction studies (NCS), a typical presentation with symmetrical involvement of all four limbs, as well as higher frequencies of elevated protein levels and albuminocytologic dissociation in the cerebrospinal fluid (CSF). " 01/18/2011 - "This study characterizes for the first time the abnormalities of nodes of Ranvier in CIAP and CIDP, and the altered expression and distribution of nodal and paranodal proteins. " 01/01/1996 - "The results of this study confirm that an elevated CSF protein and low CSF cell count are features highly supportive of the diagnosis of both AIDP and CIDP. " 01/01/2023 - "CIDP should be considered in any patient with new progressive vision loss when associated with peripheral sensorimotor symptoms and elevated CSF protein. " 12/01/2021 - "No differences in CIDP subtype, rates of cerebrospinal fluid protein level elevation, disability, or likelihood of treatment response, were observed. "
10.	Immunosuppressive Agents (Immunosuppressants)IBA 01/01/2007 - "A number of chemotherapeutic and immunosuppressive agents have also shown to be effective in treating CIDP but significant evidence is lacking; therefore, these agents are primarily used in conjunction with other modalities. " 09/01/2018 - "Two subgroups of patients frequently treated with maintenance immunosuppressants were those with improving or stable disease following first-line treatment (12, 57%) and those with progressive form of CIDP (2, 10%). " 11/01/2023 - "The inclusion criteria in this study for patients with CIDP were a diagnostic code of G61.8 in the seventh and eighth revision of the Korean Standard Classification of Disease and a >3-month history of oral immunosuppressant use. " 10/01/2016 - "It will be larger than any trial of an immunosuppressant agent so far performed in CIDP. " 01/01/2021 - "The variants of chronic inflammatory demyelinating polyneuropathy (CIDP) differ not just in their clinical, pathological and electrophysiological characteristics, but often in their indifferent response to conventional immunosuppressive agents which are effective in typical CIDP. "

Therapies and Procedures

1.	Therapeutics 03/01/2014 - "Long-term monthly IVMP pulse therapy seems to be very effective in the treatment of CIDP." 09/01/2010 - "We report 3 cases of CIDP in children with good outcome after MP pulse therapy. " 01/01/2020 - "An improved understanding of pathogenic mechanisms in CIDP will form the basis for more effective mechanism-based therapies." 04/01/2006 - "The growing body of knowledge on the pathogenesis of CIDP and clinical or electrophysiological differentiation of subforms may help to develop more effective therapies for CIDP in the next few years." 06/01/2005 - "The growing body of knowledge on the pathogenesis of CIDP and further diagnostic differentiation of subforms may help to develop more-effective therapies for CIDP in the next few years."
2.	Plasma Exchange 08/25/2015 - "Moderate- to high-quality evidence from two small trials shows that plasma exchange provides significant short-term improvement in disability, clinical impairment, and motor nerve conduction velocity in CIDP but rapid deterioration may occur afterwards. " 09/12/2012 - "Moderate to high quality evidence from two small trials showed that plasma exchange provides significant short-term improvement in disability, clinical impairment and motor nerve conduction velocity in CIDP but rapid deterioration may occur afterwards. " 10/01/2013 - "It is as effective as plasma exchange in GBS and CIDP. " 05/01/1985 - "Results in this unselected, consecutive patient trial suggest a temporal relationship between plasma exchange and clinical improvement in some patients with progressive CIDP." 01/01/1982 - "It is too early to draw any conclusion from these studies other than that the double-blind study employed here is feasible and should provide information on the efficacy of plasma exchange in CIDP if its effect is prompt and large. "
3.	Plasmapheresis 03/01/1998 - "We were not able to determine whether immunoadsorption plasmapheresis or double-filtration plasmapheresis was more effective for the patients with CIDP. " 08/01/1998 - "We conclude that DFPP was useful for the subgroups of CIDP patients, but the underlying immuno-pathological background that determine the efficacy of plasmapheresis should be elucidated." 08/05/2022 - "This study aims to compare the evolution of peripheral lymphocyte profiles in patients with CIDP according to their treatment (single centrifugation plasmapheresis or DFPP) to better grasp the action mechanisms of both techniques. " 07/01/2007 - "This review evaluates the role of plasmapheresis in CIDP." 07/01/2007 - "Plasmapheresis for chronic inflammatory demyelinating polyradiculoneuropathy."
4.	Immunotherapy 01/01/2009 - "Patients with diabetes with CIDP significantly improved after immunotherapy and during follow-up. " 02/01/2014 - "Recently, it was reported that diabetic radiculoplexus neuropathies as well as CIDP respond favorably to immunotherapy. " 04/15/2007 - "The results of the present study suggest that subclinical CNS involvement in CIDP is not uncommon in Japanese patients and that CIDP with subclinical CNS involvement is more demyelinative thus responsive to immunotherapies while those without have more axonal damage and less responsive to immunotherapies." 01/01/2006 - "Neurophysiologically probable CIDP patients should be given a trial of immunotherapy." 01/01/2023 - "Repurposing MS immunotherapies for CIDP and other autoimmune neuropathies: unfulfilled promise or efficient strategy?"
5.	Hematopoietic Stem Cell Transplantation 01/01/2023 - "A systematic review and a meta-analysis of the efficacy of hematopoietic stem cell transplantation (HSCT) treatment in refractory CIDP patients was performed. " 01/01/2023 - "Efficacy of hematopoietic stem cell transplantation treatment in refractory chronic inflammatory demyelinating polyradiculoneuropathy: a systematic review and meta-analysis." 08/01/2007 - "CIDP cured by allogeneic hematopoietic stem cell transplantation." 11/01/2021 - "To report five cases of treatment-refractory disabling CIDP treated with autologous hematopoietic stem cell transplantation (AHSCT). " 11/01/2021 - "Autologous Hematopoietic Stem Cell Transplantation for Chronic Inflammatory Demyelinating Polyradiculoneuropathy."