scholarly journals Tacrolimus Significantly Improved the Outcome of CIDP Patients With Autoantibodies Against Paranodal Proteins

2021 ◽  
Author(s):  
Meng-ge Yang ◽  
Suqiong Ji ◽  
Li Xu ◽  
Huajie Gao ◽  
Qing Zhang ◽  
...  
Keyword(s):  
Poor Prognosis ◽  
Nerve Conduction Velocity ◽  
Chronic Inflammatory Demyelinating Polyneuropathy ◽  
Nerve Repair ◽  
Immunosuppressive Agents ◽  
First Episode ◽  
High Efficacy ◽  
Immune Mediated ◽  
Inflammatory Demyelinating Polyneuropathy ◽  
Low Prevalence

Abstract Chronic inflammatory demyelinating polyneuropathy (CIDP) is an immune-mediated disabling neuropathy. CIDP with antibodies against the paranodal proteins has a low prevalence, poor prognosis and treatment difficulty. Moreover, there is currently a lack of data on the application of immunosuppressive agents. In this retrospective observational study, we aimed to evaluated the response to tacrolimus (a immunosuppressor with high efficacy) in CIDP with autoantibodies against paranodal proteins by reviewing 5 patients received daily tacrolimus. One patient started tacrolimus treatment at the first episode and eventually achieved full clinical remission without relapse. Four patients experienced worsening during corticosteroids tapering. They gradually improved after adding oral tacrolimus except one patient relapsed again due to tacrolimus discontinuation. Moreover, 3 patients were successfully withdrawn from corticosteroids and 2 patients took corticosteroids at low maintenance dose (10mg/d) after tacrolimus treatment. Furthermore, decreased nerve conduction velocity and action potential amplitudes gradually returned to a normal level after tacrolimus and corticosteroids treatment. In conclusions, tacrolimus significantly improved the outcome of CIDP patients with autoantibodies against paranodal proteins by preventing relapses, reducing corticosteroids dependence and enhancing nerve repair. Combination therapy with corticosteroids and tacrolimus may be an effective therapeutic regimen.

The Application of Clinical, Electrophysiological and Nerve Ultrasound Parameters in Distinguishing Acute-onset Chronic from Acute Inflammatory Demyelinating Polyneuropathy

2015 ◽  
Vol 10 (01) ◽  
pp. 85 ◽  
Author(s):  
Antonios Kerasnoudis ◽  
Kallia Pitarokoili ◽  
Ralf Gold ◽  
Min-Suk Yoon ◽  
◽  
...  
Keyword(s):  
Chronic Inflammatory Demyelinating Polyneuropathy ◽  
Acute Onset ◽  
Demyelinating Polyneuropathy ◽  
Nerve Ultrasound ◽  
Diagnostic Challenge ◽  
Early Application ◽  
Immune Mediated ◽  
Inflammatory Demyelinating Polyneuropathy ◽  
Ultrasound Parameters

History-taking and nerve conduction studies are fundamental for the diagnosis and assessment of the severity of acute (AIDP) or chronic inflammatory demyelinating polyneuropathy (CIDP). The diagnostic challenge of distinguishing these two immune-mediated subacute polyradiculoneuropathies remains high, as intravenous immunoglobulin and steroids exert short-term clinical improvement in the majority of the CIDP cases, whereas steroids have no effect on AIDP patients. Accordingly, the precise classification of subacute polyradiculoneuropathies significantly affects the early application of steroids in CIDP. This review aims to give a timely update on the application of clinical, electrophysiological and nerve ultrasound parameters in distinguishing subacute CIDP from AIDP.

scholarly journals Pathophysiology of the Different Clinical Phenotypes of Chronic Inflammatory Demyelinating Polyradiculoneuropathy (CIDP)

2021 ◽  
Vol 23 (1) ◽  
pp. 179
Author(s):  
Edyta Dziadkowiak ◽  
Marta Waliszewska-Prosół ◽  
Marta Nowakowska-Kotas ◽  
Sławomir Budrewicz ◽  
Zofia Koszewicz ◽  
...  
Keyword(s):  
Chronic Inflammatory Demyelinating Polyneuropathy ◽  
Relevant Literature ◽  
Chronic Inflammatory Demyelinating Polyradiculoneuropathy ◽  
Rapid Progress ◽  
Ongoing Research ◽  
Clinical Phenotypes ◽  
Current State ◽  
Immune Mediated ◽  
Key Points ◽  
Inflammatory Demyelinating Polyneuropathy

Chronic inflammatory demyelinating polyneuropathy (CIDP) is the most common form of autoimmune polyneuropathy. It is a chronic disease and may be monophasic, progressive or recurrent with exacerbations and incomplete remissions, causing accumulating disability. In recent years, there has been rapid progress in understanding the background of CIDP, which allowed us to distinguish specific phenotypes of this disease. This in turn allowed us to better understand the mechanism of response or non-response to various forms of therapy. On the basis of a review of the relevant literature, the authors present the current state of knowledge concerning the pathophysiology of the different clinical phenotypes of CIDP as well as ongoing research in this field, with reference to key points of immune-mediated processes involved in the background of CIDP.

CIDP and Related Variants and Overlap Disorders

2019 ◽  
pp. 208-244
Author(s):  
Jeffrey A. Allen
Keyword(s):  
Monoclonal Gammopathy ◽  
Chronic Inflammatory Demyelinating Polyneuropathy ◽  
Clinical Manifestations ◽  
Multifocal Motor Neuropathy ◽  
Motor Neuropathy ◽  
Peripheral Nerve Disorder ◽  
Diagnostic Data ◽  
Immune Mediated ◽  
Inflammatory Demyelinating Polyneuropathy ◽  
History Of

This chapter begins with a history of chronic immunological neuropathies. It then looks in particular at chronic inflammatory demyelinating polyneuropathy (CIDP), which is an immune-mediated peripheral nerve disorder characterized by progressive or relapsing motor or sensory symptoms. It then considers the epidemiology, clinical manifestations, and electrophysiology of CIDP. The chapter examines diagnostic data and diagnostic criteria for CIDP. It then looks at other neuropathies with clinical and electrophysiologic features that are shared with CIDP. Particular attention is given to neuropathy associated with monoclonal gammopathy including IgM associated neuropathy and POEMS syndrome, polyneuropathies associated with specific autoantibodies including antibodies that target nodal and paranodal structures, and multifocal motor neuropathy. For each condition diagnostic data, pathophysiology and treatment are discussed.

scholarly journals Chronic inflammatory demyelinating polyneuropathy: update on diagnosis, immunopathogenesis and treatment

2019 ◽  
Vol 90 (9) ◽  
pp. 981-987 ◽  
Author(s):  
Helmar Christoph Lehmann ◽  
David Burke ◽  
Satoshi Kuwabara
Keyword(s):  
Chronic Inflammatory Demyelinating Polyneuropathy ◽  
Demyelinating Polyneuropathy ◽  
Motor Neuropathy ◽  
Immune Mediated ◽  
Inflammatory Demyelinating Polyneuropathy ◽  
Costly Treatment ◽  
Relative Rarity ◽  
Treatment Responses ◽  
Immunomodulating Drugs ◽  
Made In

Chronic inflammatory demyelinating polyneuropathy (CIDP) is an immune-mediated neuropathy typically characterised by symmetrical involvement, and proximal as well as distal muscle weakness (typical CIDP). However, there are several ‘atypical’ subtypes, such as multifocal acquired demyelinating sensory and motor neuropathy (Lewis-Sumner syndrome) and ‘distal acquired demyelinating symmetric neuropathy’, possibly having different immunopathogenesis and treatment responses. In the absence of diagnostic and pathogenetic biomarkers, diagnosis and treatment may be difficult, but recent progress has been made in the application of neuroimaging tools demonstrating nerve hypertrophy and in identifying subgroups of patients who harbour antibodies against nodal proteins such as neurofascin and contactin-1. Despite its relative rarity, CIDP represents a significant economic burden, mostly due to costly treatment with immunoglobulin. Recent studies have demonstrated the efficacy of subcutaneous as well as intravenous immunoglobulin as maintenance therapy, and newer immunomodulating drugs can be used in refractory cases. This review provides an overview focusing on advances over the past several years.

scholarly journals A Severe Course of Relapsing-Remitting Acute-Onset Chronic Inflammatory Demyelinating Polyneuropathy in a Young Man

2021 ◽  
pp. 72-76
Author(s):  
Anna P. Patnaik ◽  
Joseph Mininni ◽  
Neil C. Porter ◽  
Nicholas A. Morris
Keyword(s):  
Chronic Inflammatory Demyelinating Polyneuropathy ◽  
Severe Disease ◽  
Acute Onset ◽  
Demyelinating Polyneuropathy ◽  
Immune Mediated ◽  
Relapsing Remitting ◽  
Inflammatory Demyelinating Polyneuropathy ◽  
High Concentrations ◽  
Guillain Barré

Acute-onset chronic inflammatory demyelinating polyneuropathy (A-CIDP) is an immune mediated neuropathy characterized by progressive weakness and sensory impairment lasting over 2 months. Guillain-Barré-Strohl syndrome (GBS) is an immune mediated polyneuropathy with a similar presentation often over less than 4 weeks. While some have argued for the existence of recurrent GBS, most classify the syndrome as a form of relapsing-remitting CIDP. However, there are cases of GBS with treatment-related fluctuations that must be distinguished from A-CIDP as patients with A-CIDP require long-term immunotherapy. In this case report, we discuss a patient with multiple relapses over 3 years, who is more likely to have A-CIDP. His ganglioside profile, which has rarely been reported in A-CIDP, included high concentrations of anti-GM1, anti-GD1a, and anti-GD1b antibodies, which may account for his severe disease course.

scholarly journals AN UPDATE DATA ON THE TREATMENT OF CHRONIC INFLAMMATORY DEMYELINATING POLYNEUROPATHY (review of literature)

2019 ◽  
Vol 26 (1) ◽  
pp. 9-19
Author(s):  
Roman A. Gapeshin ◽  
Evgeny R. Barantsevich ◽  
Dmitry I. Rudenko ◽  
Oksana V. Posokhina ◽  
Tima R. Stuchevskaya
Keyword(s):  
Monoclonal Antibodies ◽  
Peripheral Neuropathy ◽  
Plasma Exchange ◽  
Chronic Inflammatory Demyelinating Polyneuropathy ◽  
Mechanisms Of Action ◽  
Demyelinating Polyneuropathy ◽  
Review Of Literature ◽  
Advantages And Disadvantages ◽  
Immune Mediated ◽  
Inflammatory Demyelinating Polyneuropathy

Chronic inflammatory demyelinating polyneuropathy (CIDP) is a heterogeneous immune-mediated peripheral neuropathy with progressive or relapse-remitting course. Incidence of CIDP ranged between 1 and 8.9/100 000. Recently, most frequent therapies for CIDP treatment was glucocorticosteroids, intravenous immunoglobulin and plasma exchange. In cases of ineffectiveness or lack of effectiveness, cytostatics, monoclonal antibodies and others could be used for CIDP treatment. In the article, authors presented an update data on the use of main methods for CIDP therapy, their mechanisms of action, indication for their use and advantages and disadvantages of each of them.

Sign in / Sign up

Export Citation Format

Share Document