Monitoring Clinical Course and Treatment Response in Chronic Inflammatory Demyelinating Polyneuropathy During Routine Care

Allen, Jeffrey A.; Merkies, Ingemar S. J.; Lewis, Richard A.

doi:10.1001/jamaneurol.2020.0781

Cited by 27 publications

(26 citation statements)

References 62 publications

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“…Recognizing that CIDP disease monitoring is best done from a multimodality approach, we also encourage collection of disability outcomes concomitantly with grip strength. 20 Although some training is required, the I-RODS and ONLS (as well as the closely related Inflammatory Neuropathy Cause and Treatment scale 21 ) are easy to learn and equally as feasible for patients to complete at home on their own. Strength assessment with handheld dynamometer and disability assessment with one of the validated patient-reported disability scales are well poised to objectify disease progression and treatment response in patients with CIDP even without an inperson encounter.…”

Section: Discussionmentioning

confidence: 99%

Quantifying Treatment-Related Fluctuations in CIDP

et al. 2021

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ObjectiveThe objective of this study was to explore the extent of IV immunoglobulin (IVIG) treatment-related fluctuations (TRFs) by using home collection of daily grip strength in patients with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) and to use that information to develop evidence-based treatment optimization strategies.MethodsThis prospective observational study included 25 patients with well-defined CIDP. Participants recorded grip strength daily for 6 months. Disability and gait metrics were collected weekly. Serum immunoglobulin G levels were obtained at peak, trough, and midcycle IVIG intervals. Day-to-day grip strength changes <10% were considered random. To identify patients with TRFs, 3-day averaged grip strength was calculated on each consecutive day after an IVIG infusion. TRFs were defined as ≥10% 3-day averaged grip strength difference compared to the pre-IVIG baseline.ResultsParticipants successfully recorded grip strength on all but 9% of recordable days. Twelve patients (48%) were classified as low/no fluctuaters and 13 (52%) as frequent fluctuaters. In the frequent fluctuating group, grip strength improved over 1 week and thereafter was relatively stable until the third week after infusion. Grip strength was significantly correlated with measures of disability.ConclusionsGrip strength collection by patients at home is reliable, valid, and feasible. A change in grip strength by ≥10% is a useful, practical, and evidence-based approach that may be used to identify clinically meaningful TRFs. From these data, we propose a treatment optimization strategy for patients with CIDP on chronic IVIG that may be applied to routine clinic care during both face-to-face and virtual video or telephone patient encounters.Trial Registration InformationClinicalTrials.gov Identifier: NCT02414490.

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Section: Discussionmentioning

confidence: 99%

Quantifying Treatment-Related Fluctuations in CIDP

et al. 2021

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“…Our study used three different outcome measures along with MCID based cut-off values to define improvement. We recognize that these criteria do not always reflect clinical practice and that a less static, more patient-tailored assessment may be more suitable to define meaningful improvement and treatment (non) responders [47,[49][50][51]. We advocate a multimodal approach in those patients with expected limited improvement, for example in patients with severe axonal damage at presentation or those with only minimal disability or impairment, if treatment is considered justified at all.…”

Section: Discussionmentioning

confidence: 99%

Clinical outcome of CIDP one year after start of treatment: a prospective cohort study

et al. 2021

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Objective To assess clinical outcome in treatment-naive patients with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). Methods We included adult treatment-naive patients participating in the prospective International CIDP Outcome Study (ICOS) that fulfilled the European Federation of Neurological Societies/Peripheral Nerve Society (EFNS/PNS) diagnostic criteria for CIDP. Patients were grouped based on initial treatment with (1) intravenous immunoglobulin (IVIg), (2) corticosteroid monotherapy or (3) IVIg and corticosteroids (combination treatment). Outcome measures included the inflammatory Rasch-built overall disability scale (I-RODS), grip strength, and Medical Research Council (MRC) sum score. Treatment response, treatment status, remissions (improved and untreated), treatment changes, and residual symptoms or deficits were assessed at 1 year. Results Forty patients were included of whom 18 (45%) initially received IVIg, 6 (15%) corticosteroids, and 16 (40%) combination treatment. Improvement on ≥ 1 of the outcome measures was seen in 31 (78%) patients. At 1 year, 19 (48%) patients were still treated and fourteen (36%) patients were in remission. Improvement was seen most frequently in patients started on IVIg (94%) and remission in those started on combination treatment (44%). Differences between groups did not reach statistical significance. Residual symptoms or deficits ranged from 25% for neuropathic pain to 96% for any sensory deficit. Conclusions Improvement was seen in most patients. One year after the start of treatment, more than half of the patients were untreated and around one-third in remission. Residual symptoms and deficits were common regardless of treatment.

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“…Over the last decade, renewed interest has focused on capturing clinical outcome using multiple modalities for research trials in CIDP. A combined set of outcome measures for CIDP trials and clinical evaluation emerged, incorporating assessment of: (a) disability (Inflammatory Rasch‐built Overall Disability Scale [I‐RODS] and Inflammatory Neuropathy Cause and Treatment [INCAT]); (b) strength (grip strength testing, manual muscle testing, Medical Research Council summated score); (c) gait assessment (timed up‐and‐go test); and (d) quality‐of‐life measures (EuroQoL 5‐Dimension Questionnaire, Patient Global Impression of Change) 90,91 . Although this approach applies to research trials, it is equally important in the clinical setting.…”

Section: Outcome Measuresmentioning

confidence: 99%

Chronic inflammatory demyelinating polyradiculoneuropathy—Diagnostic pitfalls and treatment approach

2020

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Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is characterized by progressive weakness and sensory loss, often affecting patientsʼ ability to walk and perform activities of daily living independently. With the lack of a diagnostic biomarker, the diagnosis relies on clinical suspicion, clinical findings, and the demonstration of demyelinating changes on electrodiagnostic (EDx) testing and nerve pathology. As a result, patients can often be misdiagnosed with CIDP and unnecessarily treated with immunotherapy. Interpreting the EDx testing and cerebrospinal fluid findings in light of the clinical phenotype, recognizing atypical forms of CIDP, and screening for CIDP mimickers are the mainstays of the approach to patients suspected of having CIDP, and are detailed in this review. We also review the currently available treatment options, including intravenous immunoglobulin (IVIg), corticosteroids (CCS), and plasma exchange (PE), and discuss how to approach treatment‐refractory cases. Finally, we emphasize the need to adopt objective outcome measures to monitor treatment response.

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Monitoring Clinical Course and Treatment Response in Chronic Inflammatory Demyelinating Polyneuropathy During Routine Care

Cited by 27 publications

References 62 publications

Quantifying Treatment-Related Fluctuations in CIDP

Quantifying Treatment-Related Fluctuations in CIDP

Clinical outcome of CIDP one year after start of treatment: a prospective cohort study

Chronic inflammatory demyelinating polyradiculoneuropathy—Diagnostic pitfalls and treatment approach

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