Characteristics and outcome of chronic inflammatory demyelinating polyradiculoneuropathy patients according to their diagnostic certainty based on the 2021 EAN/PNS criteria
To describe the clinical characteristics and long term outcome of CIDP patients according to 2021 EAN/PNS diagnostic certainty categories. We reviewed clinical data, response to treatment, cerebrospinal fluid examination, and nerve conduction studies parameters of 39 adult “CIDP” and 24 “possible CI...
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| Vydáno v: | Journal of the neurological sciences Ročník 466; s. 123259 |
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Elsevier B.V
15.11.2024
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| Abstract | To describe the clinical characteristics and long term outcome of CIDP patients according to 2021 EAN/PNS diagnostic certainty categories.
We reviewed clinical data, response to treatment, cerebrospinal fluid examination, and nerve conduction studies parameters of 39 adult “CIDP” and 24 “possible CIDP” patients. Data were collected at diagnosis and after one (T1), two (T2), three (T3) and five years (T5).
At diagnosis, “possible CIDP” patients' phenotypes were more atypical (especially focal/multifocal, p < .01) and “CIDP” patients had a higher NIS and INCAT scores (p = .08 and 0.08). Compared to baseline: median NIS score decreased in “CIDP” and was stable in “possible CIDP” patients at T1 (p < .05), T2 (p < .05) and T3 (p < .01); median MRC score slightly increased in “CIDP” and was stable in “possible CIDP” patients at T2 (p < .05); and INCAT disability scale slightly decreased in “CIDP” and was stable in “possible CIDP” patients at T3 (p < .05). The proportion of moderate to severely disabled (mRS > 2) patients in “possible CIDP” group was higher than in “CIDP” group (not significant). “CIDP” patients had a better objective response to immunotherapy (59 % responders) than “possible CIDP” patients (29 % responders, p < .05), especially among typical CIDP patients (86 % of responders in “CIDP” versus 33 % of responders in “possible CIDP” patients, p < .05).
“CIDP” patients had a more severe neuropathy, estimated with the NIS and INCAT scores, and “possible CIDP” patients had a more atypical phenotype at baseline. Our data suggest that long-term patient outcome and response to immunotherapy is better in “CIDP” than “possible CIDP”.
•EAN/PNS 2021 classify patients as “CIDP” or “possible CIDP”.•“CIDP” patients respond better to immunotherapy than “possible CIDP” patients.•Long term outcome seems better in “CIDP” patients.•“Possible CIDP”: axonal loss, misdiagnosis, diagnostic delay may explain poor outcome. |
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| AbstractList | To describe the clinical characteristics and long term outcome of CIDP patients according to 2021 EAN/PNS diagnostic certainty categories.
We reviewed clinical data, response to treatment, cerebrospinal fluid examination, and nerve conduction studies parameters of 39 adult “CIDP” and 24 “possible CIDP” patients. Data were collected at diagnosis and after one (T1), two (T2), three (T3) and five years (T5).
At diagnosis, “possible CIDP” patients' phenotypes were more atypical (especially focal/multifocal, p < .01) and “CIDP” patients had a higher NIS and INCAT scores (p = .08 and 0.08). Compared to baseline: median NIS score decreased in “CIDP” and was stable in “possible CIDP” patients at T1 (p < .05), T2 (p < .05) and T3 (p < .01); median MRC score slightly increased in “CIDP” and was stable in “possible CIDP” patients at T2 (p < .05); and INCAT disability scale slightly decreased in “CIDP” and was stable in “possible CIDP” patients at T3 (p < .05). The proportion of moderate to severely disabled (mRS > 2) patients in “possible CIDP” group was higher than in “CIDP” group (not significant). “CIDP” patients had a better objective response to immunotherapy (59 % responders) than “possible CIDP” patients (29 % responders, p < .05), especially among typical CIDP patients (86 % of responders in “CIDP” versus 33 % of responders in “possible CIDP” patients, p < .05).
“CIDP” patients had a more severe neuropathy, estimated with the NIS and INCAT scores, and “possible CIDP” patients had a more atypical phenotype at baseline. Our data suggest that long-term patient outcome and response to immunotherapy is better in “CIDP” than “possible CIDP”.
•EAN/PNS 2021 classify patients as “CIDP” or “possible CIDP”.•“CIDP” patients respond better to immunotherapy than “possible CIDP” patients.•Long term outcome seems better in “CIDP” patients.•“Possible CIDP”: axonal loss, misdiagnosis, diagnostic delay may explain poor outcome. To describe the clinical characteristics and long term outcome of CIDP patients according to 2021 EAN/PNS diagnostic certainty categories.INTRODUCTIONTo describe the clinical characteristics and long term outcome of CIDP patients according to 2021 EAN/PNS diagnostic certainty categories.We reviewed clinical data, response to treatment, cerebrospinal fluid examination, and nerve conduction studies parameters of 39 adult "CIDP" and 24 "possible CIDP" patients. Data were collected at diagnosis and after one (T1), two (T2), three (T3) and five years (T5).METHODSWe reviewed clinical data, response to treatment, cerebrospinal fluid examination, and nerve conduction studies parameters of 39 adult "CIDP" and 24 "possible CIDP" patients. Data were collected at diagnosis and after one (T1), two (T2), three (T3) and five years (T5).At diagnosis, "possible CIDP" patients' phenotypes were more atypical (especially focal/multifocal, p < .01) and "CIDP" patients had a higher NIS and INCAT scores (p = .08 and 0.08). Compared to baseline: median NIS score decreased in "CIDP" and was stable in "possible CIDP" patients at T1 (p < .05), T2 (p < .05) and T3 (p < .01); median MRC score slightly increased in "CIDP" and was stable in "possible CIDP" patients at T2 (p < .05); and INCAT disability scale slightly decreased in "CIDP" and was stable in "possible CIDP" patients at T3 (p < .05). The proportion of moderate to severely disabled (mRS > 2) patients in "possible CIDP" group was higher than in "CIDP" group (not significant). "CIDP" patients had a better objective response to immunotherapy (59 % responders) than "possible CIDP" patients (29 % responders, p < .05), especially among typical CIDP patients (86 % of responders in "CIDP" versus 33 % of responders in "possible CIDP" patients, p < .05).RESULTSAt diagnosis, "possible CIDP" patients' phenotypes were more atypical (especially focal/multifocal, p < .01) and "CIDP" patients had a higher NIS and INCAT scores (p = .08 and 0.08). Compared to baseline: median NIS score decreased in "CIDP" and was stable in "possible CIDP" patients at T1 (p < .05), T2 (p < .05) and T3 (p < .01); median MRC score slightly increased in "CIDP" and was stable in "possible CIDP" patients at T2 (p < .05); and INCAT disability scale slightly decreased in "CIDP" and was stable in "possible CIDP" patients at T3 (p < .05). The proportion of moderate to severely disabled (mRS > 2) patients in "possible CIDP" group was higher than in "CIDP" group (not significant). "CIDP" patients had a better objective response to immunotherapy (59 % responders) than "possible CIDP" patients (29 % responders, p < .05), especially among typical CIDP patients (86 % of responders in "CIDP" versus 33 % of responders in "possible CIDP" patients, p < .05)."CIDP" patients had a more severe neuropathy, estimated with the NIS and INCAT scores, and "possible CIDP" patients had a more atypical phenotype at baseline. Our data suggest that long-term patient outcome and response to immunotherapy is better in "CIDP" than "possible CIDP".CONCLUSION"CIDP" patients had a more severe neuropathy, estimated with the NIS and INCAT scores, and "possible CIDP" patients had a more atypical phenotype at baseline. Our data suggest that long-term patient outcome and response to immunotherapy is better in "CIDP" than "possible CIDP". To describe the clinical characteristics and long term outcome of CIDP patients according to 2021 EAN/PNS diagnostic certainty categories. We reviewed clinical data, response to treatment, cerebrospinal fluid examination, and nerve conduction studies parameters of 39 adult "CIDP" and 24 "possible CIDP" patients. Data were collected at diagnosis and after one (T1), two (T2), three (T3) and five years (T5). At diagnosis, "possible CIDP" patients' phenotypes were more atypical (especially focal/multifocal, p < .01) and "CIDP" patients had a higher NIS and INCAT scores (p = .08 and 0.08). Compared to baseline: median NIS score decreased in "CIDP" and was stable in "possible CIDP" patients at T1 (p < .05), T2 (p < .05) and T3 (p < .01); median MRC score slightly increased in "CIDP" and was stable in "possible CIDP" patients at T2 (p < .05); and INCAT disability scale slightly decreased in "CIDP" and was stable in "possible CIDP" patients at T3 (p < .05). The proportion of moderate to severely disabled (mRS > 2) patients in "possible CIDP" group was higher than in "CIDP" group (not significant). "CIDP" patients had a better objective response to immunotherapy (59 % responders) than "possible CIDP" patients (29 % responders, p < .05), especially among typical CIDP patients (86 % of responders in "CIDP" versus 33 % of responders in "possible CIDP" patients, p < .05). "CIDP" patients had a more severe neuropathy, estimated with the NIS and INCAT scores, and "possible CIDP" patients had a more atypical phenotype at baseline. Our data suggest that long-term patient outcome and response to immunotherapy is better in "CIDP" than "possible CIDP". AbstractIntroductionTo describe the clinical characteristics and long term outcome of CIDP patients according to 2021 EAN/PNS diagnostic certainty categories. MethodsWe reviewed clinical data, response to treatment, cerebrospinal fluid examination, and nerve conduction studies parameters of 39 adult “CIDP” and 24 “possible CIDP” patients. Data were collected at diagnosis and after one (T1), two (T2), three (T3) and five years (T5). ResultsAt diagnosis, “possible CIDP” patients' phenotypes were more atypical (especially focal/multifocal, p < .01) and “CIDP” patients had a higher NIS and INCAT scores ( p = .08 and 0.08). Compared to baseline: median NIS score decreased in “CIDP” and was stable in “possible CIDP” patients at T1 ( p < .05), T2 (p < .05) and T3 (p < .01); median MRC score slightly increased in “CIDP” and was stable in “possible CIDP” patients at T2 (p < .05); and INCAT disability scale slightly decreased in “CIDP” and was stable in “possible CIDP” patients at T3 (p < .05). The proportion of moderate to severely disabled (mRS > 2) patients in “possible CIDP” group was higher than in “CIDP” group (not significant). “CIDP” patients had a better objective response to immunotherapy (59 % responders) than “possible CIDP” patients (29 % responders, p < .05), especially among typical CIDP patients (86 % of responders in “CIDP” versus 33 % of responders in “possible CIDP” patients, p < .05). Conclusion“CIDP” patients had a more severe neuropathy, estimated with the NIS and INCAT scores, and “possible CIDP” patients had a more atypical phenotype at baseline. Our data suggest that long-term patient outcome and response to immunotherapy is better in “CIDP” than “possible CIDP”. |
| ArticleNumber | 123259 |
| Author | Staedler, Katia Vicino, Alex Loser, Valentin Kuntzer, Thierry Théaudin, Marie |
| Author_xml | – sequence: 1 givenname: Valentin surname: Loser fullname: Loser, Valentin email: valentin.loser@chuv.ch – sequence: 2 givenname: Alex surname: Vicino fullname: Vicino, Alex email: alex.vicino@chuv.ch – sequence: 3 givenname: Katia surname: Staedler fullname: Staedler, Katia email: katia.staedler@chuv.ch – sequence: 4 givenname: Thierry surname: Kuntzer fullname: Kuntzer, Thierry email: thierry.kuntzer@chuv.ch – sequence: 5 givenname: Marie surname: Théaudin fullname: Théaudin, Marie email: marie.theaudin@chuv.ch |
| BackLink | https://www.ncbi.nlm.nih.gov/pubmed/39388750$$D View this record in MEDLINE/PubMed |
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| Cites_doi | 10.1002/mus.27820 10.1111/jns5.12038 10.1586/ern.09.30 10.1002/mus.26772 10.1016/j.clinph.2020.12.017 10.1002/mus.880141111 10.1111/jns.12470 10.1136/jnnp-2018-318714 10.1002/mus.21733 10.1111/ene.14796 10.1159/000052074 10.1002/mus.27722 10.1111/jns.12302 10.1111/ene.15156 10.1007/s00415-018-09174-z 10.1016/j.jneuroim.2014.03.002 10.1111/ene.14101 10.1016/j.clinph.2015.05.004 10.1002/mus.22038 10.1212/01.WNL.0000158680.89323.F8 10.1136/jnnp.2004.056515 10.1136/jnnp.72.5.596 10.1111/j.1529-8027.2010.00251.x 10.1136/jnnp-2022-329357 10.1111/jns.12455 10.1371/journal.pone.0139674 10.1002/mus.26488 10.1136/jnnp-2013-306105 10.1212/01.wnl.0000260972.07422.ea |
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We reviewed clinical... AbstractIntroductionTo describe the clinical characteristics and long term outcome of CIDP patients according to 2021 EAN/PNS diagnostic certainty categories.... To describe the clinical characteristics and long term outcome of CIDP patients according to 2021 EAN/PNS diagnostic certainty categories.INTRODUCTIONTo... |
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| Title | Characteristics and outcome of chronic inflammatory demyelinating polyradiculoneuropathy patients according to their diagnostic certainty based on the 2021 EAN/PNS criteria |
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