Long‐term outcome in chronic inflammatory demyelinating polyneuropathy patients treated with intravenous immunoglobulin: A retrospective study

Querol, Luís; Rojas‐García, Ricard; Casasnovas, Carlos; Sedano, María J.; Muñoz-Blanco, José Luís; Alberti, M.; Paradas, Carmen; Sevilla, Teresa; Pardo, Julio; Capablo, J.L.; Sivera, Rafael; Guerrero, Antonio; Gutiérrez-Rivas, Eduardo; Illa, Isabel

doi:10.1002/mus.23843

Cited by 46 publications

(49 citation statements)

References 23 publications

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“…This was much below the percentage of responsive patients in our negative group (59%) or in reported studies (54%-63%). 1,[22][23][24] Similar observations were reported by a Spanish group, in which none of the 4 patients responded to IVIg. 3 In contrast, beneficial response was noted after plasma exchange in another study.…”

Section: Association Of Cidp With Anti-nf155 Igg4 Antibodiessupporting

confidence: 86%

Neurofascin-155 IgG4 in chronic inflammatory demyelinating polyneuropathy

et al. 2016

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Objective: We report the clinical and serologic features of Japanese patients with chronic inflammatory demyelinating polyneuropathy (CIDP) displaying anti-neurofascin-155 (NF155) immunoglobulin G4 (IgG4) antibodies. Methods:In sera from 533 patients with CIDP, anti-NF155 IgG4 antibodies were detected by ELISA. Binding of IgG antibodies to central and peripheral nerves was tested.Results: Anti-NF155 IgG4 antibodies were identified in 38 patients (7%) with CIDP, but not in disease controls or normal participants. These patients were younger at onset as compared to 100 anti-NF155-negative patients with CIDP. Twenty-eight patients (74%) presented with sensory ataxia, 16 (42%) showed tremor, 5 (13%) presented with cerebellar ataxia associated with nystagmus, 3 (8%) had demyelinating lesions in the CNS, and 20 of 25 (80%) had poor response to IV immunoglobulin. The clinical features of the antibody-positive patients were statistically more frequent as compared to negative patients with CIDP (n 5 100). Anti-NF155 IgG antibodies targeted similarly central and peripheral paranodes.Conclusion: Anti-NF155 IgG4 antibodies were associated with a subgroup of patients with CIDP showing a younger age at onset, ataxia, tremor, CNS demyelination, and a poor response to IV immunoglobulin. The autoantibodies may serve as a biomarker to improve patients' diagnosis and guide treatments. Neurology ® 2016;86:800-807 GLOSSARY Caspr1 5 contactin-associated protein-1; CCPD 5 combined central and peripheral demyelination; CI 5 confidence interval; CIDP 5 chronic inflammatory demyelinating polyneuropathy; CNTN1 5 contactin 1; GBS 5 Guillain-Barré syndrome; IgG4 5 immunoglobulin G4; IVIg 5 IV immunoglobulin; MS 5 multiple sclerosis; NF155 5 neurofascin-155; OR 5 odds ratio; PNS 5 peripheral nervous system.Chronic inflammatory demyelinating polyneuropathy (CIDP) is the most common acquired immune-mediated neuropathy worldwide and is clinically heterogeneous.1 Proven treatments for CIDP include corticosteroids, plasma exchange, and IV immunoglobulin (IVIg). However, the response rates to treatments are highly heterogeneous between patients. This emphasizes that patients and clinicians require biomarkers to identify CIDP subgroups and guide specific immunotherapeutic options.Immunoglobulin G4 (IgG4) autoantibodies to neurofascin-155 (NF155) were recently documented in patients with CIDP.2,3 NF155 belongs to the L1 family of adhesion molecules and is expressed at paranodes by the terminal loops of myelin and associates with the axonal cell adhesion molecules CNTN1 and contactin-associated protein-1 (Caspr1). 4 This ternary complex of glycoproteins is required for the rapid propagation of the nerve impulses along myelinated axons. 5,6 Three of 4 patients with anti-NF155 IgG4 showed disabling tremor and all showed poor response to IVIg. 3 This suggested that IgG4 autoantibodies participate in CIDP pathogenesis; nonetheless, the low number of reactive patients precluded statistical correlation.

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Section: Association Of Cidp With Anti-nf155 Igg4 Antibodiessupporting

confidence: 86%

Neurofascin-155 IgG4 in chronic inflammatory demyelinating polyneuropathy

et al. 2016

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“…In a recent study of 86 non-treatment-naïve patients with CIDP treated with IVIg, the only variable that was associated with reaching remission (asymptomatic without treatment) at long term was a better response during the first 6 months 36. Unfortunately, some patients in this study had been treated simultaneously with other immunosuppressive agents besides IVIg 36…”

Section: Discussionmentioning

confidence: 78%

Intravenous immunoglobulin response in treatment-nai ve chronic inflammatory demyelinating polyradiculoneuropathy

Kuitwaard

Hahn

Vermeulen

et al. 2014

Journal of Neurology, Neurosurgery & Psychiatry

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“…All of these are validated scales that have been used in a variety of neuromuscular disorders and in previous studies of IBM. 26–28 Use of the armrest was allowed for the timed get up test and use of a cane or walker was allowed for the 6 min walk test. For statistical purposes any individual unable to get up from a chair, even with the use of arm rests, was scored at 20 s (maximal score) while for the 6 min walk test, a distance of 0 feet was scored for individuals who were unable to perform the test.…”

Section: Methodsmentioning

confidence: 99%

Seropositivity for NT5c1A antibody in sporadic inclusion body myositis predicts more severe motor, bulbar and respiratory involvement

Goyal

Cash

Alam

et al. 2015

J Neurol Neurosurg Psychiatry

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Objectives To explore phenotypic differences between individuals with sporadic inclusion body myositis (sIBM) who are seropositive for the NT5c1A antibody compared with those who are seronegative. Methods Cross-sectional clinical, serological and functional analysis in 25 consecutive participants with sIBM. Results All participants met criteria for clinically defined or probable sIBM. 18 of 25 participants with sIBM (72%) were seropositive for the NT5c1A antibody. No differences between median age and duration of illness between the two groups were seen. Females have higher odds of being seropositive (OR=2.30). Participants with seropositive sIBM took significantly longer to get up and stand (p=0.012). There were no significant differences between the two groups in terms of distance covered on a 6 min walk. Seropositive participants were more likely to require assistive devices such as a walker or wheelchair for mobility (OR=23.00; p=0.007). A number of secondary (exploratory) outcomes were assessed. NT5c1A seropositive sIBM cases had lower total Medical Research Council (MRC) sum score and MRC sum score on the right (p=0.03 and 0.02, respectively). Participants with the NT5c1A antibody were significantly more likely to have symptoms of dysphagia (OR=10.67; p=0.03) and reduced forced vital capacity (p=0.005). Facial weakness occurred in 50% of seropositive participants while it was only seen in 14% of seronegative participants. Conclusions Even though the small sample size limits definite conclusions, our cross-sectional study showed seropositivity to the NT5c1A antibody is associated with greater motor and functional disability in sIBM. The study also suggests more prominent bulbar, facial and respiratory involvement in individuals positive for NT5c1A antibodies.

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Long‐term outcome in chronic inflammatory demyelinating polyneuropathy patients treated with intravenous immunoglobulin: A retrospective study

Abstract: A significant percentage of patients did not require any additional drugs in the long term. This suggests that treatment effect or disease outcome may be stable over time, and treatment regimens should therefore be individualized to avoid overtreatment.

Cited by 46 publications

References 23 publications

Neurofascin-155 IgG4 in chronic inflammatory demyelinating polyneuropathy

Neurofascin-155 IgG4 in chronic inflammatory demyelinating polyneuropathy

Intravenous immunoglobulin response in treatment-nai ve chronic inflammatory demyelinating polyradiculoneuropathy

Seropositivity for NT5c1A antibody in sporadic inclusion body myositis predicts more severe motor, bulbar and respiratory involvement

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