Evaluation of patients with refractory chronic inflammatory demyelinating polyneuropathy

Kaplan, Artem; Brannagan, Thomas H.

doi:10.1002/mus.25271

Cited by 43 publications

(41 citation statements)

References 33 publications

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“…First, a substantial proportion of patients with CIDP in this study were diagnosed with an initial other disease. While previous studies indicated that patients diagnosed with CIDP in up to 54% of patients may actually have another diagnosis (CIDP overdiagnosis), our study suggest that CIDP underdiagnosis also occurs. CIDP underdiagnosis appears to be mainly related to poor or limited performance and interpretation of clinical and diagnostic assessments.…”

Section: Discussioncontrasting

confidence: 84%

Clinical factors, diagnostic delay, and residual deficits in chronic inflammatory demyelinating polyradiculoneuropathy

Bunschoten

Blomkwist-Markens²,

Horemans³

et al. 2019

J Peripheral Nervous Sys

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Management of patients with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is complicated by the challenging diagnosis, monitoring of disease activity, and treatment response. Real world data about the current practice of care in CIDP are rare, but important to improve clinical guidelines. In this study, we determined the current practice of diagnosis and treatment in relation to the clinical outcome of patients with CIDP in a cross‐sectional study in The Netherlands. All patients registered at the Dutch neuromuscular patient organization and patients at the Erasmus Medical Center were approached. CIDP diagnosis was confirmed by using patient files and expert consensus. The response rate was 137/197 (70%) and the diagnosis CIDP was confirmed in 112 patients. The time from onset of symptoms until CIDP diagnosis was median 5 months and > 12 months in 26% of the patients. Patients with a late diagnosis (>5 months) compared to patients with an early diagnosis (≤5 months) more frequently had another initial diagnosis (P < .001), multifocal abnormalities (P = .011), final diagnosis made in university hospitals (P = .001), and more (residual) complaints, also illustrated via two patient reported, validated clinical outcome measures. Although 97% of patients received treatment, 88% reported (residual) neurological symptoms and deficits. CIDP diagnosis is often delayed, especially in patients with atypical CIDP variants. Diagnostic delay may result in delayed initiation of treatment. A more rapid and accurate diagnosis of CIDP may prevent or at least reduce residual neurological deficits and accompanying disability in CIDP.

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Section: Discussioncontrasting

confidence: 84%

Clinical factors, diagnostic delay, and residual deficits in chronic inflammatory demyelinating polyradiculoneuropathy

Bunschoten

Blomkwist-Markens²,

Horemans³

et al. 2019

J Peripheral Nervous Sys

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“…Maintenance IVIG as frequently as every two weeks has been reported to be effective and well tolerated in some cases. 108 Immediate adverse reactions following IVIG administration are often mild, including transient flu-like symptoms, headache, flushing of the face, change in blood pressure, and tachycardia. Minor reactions can often be ameliorated by pretreatment with analgesics, antihistamines, and corticosteroids, and by slowing the infusion rate.…”

Section: Immunoglobulinmentioning

confidence: 99%

Chronic Inflammatory Demyelinating Polyradiculoneuropathy

Shije

Brannagan

2019

Semin Neurol

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Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is a relatively common autoimmune disorder affecting the peripheral nerves and nerve roots, often causing progressive or recurrent weakness with diminished reflexes. Electrodiagnostic (EDx) studies, cerebral spinal fluid (CSF) analysis, and nerve biopsy may help provide supportive evidence for the diagnosis. Most cases have a favorable response to one of the three first-line treatments: corticosteroids, IV immunoglobulin (IVIG) and plasmapheresis. Responses to these treatments may vary among individual patients. There is evidence that a small percentage of CIDP patients with IgG class 4 (IgG4) autoantibodies to paranodal proteins have characteristic clinical features and poorer response to IVIG. Chemotherapy and other immunomodulatory agents, as well as hematopoietic stem cell transplantation, may be considered in refractory cases. The degree of disability varies, and most patients require ongoing treatment to maintain stable disease, although long-term remission or cure may be achieved in some patients.

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“…Considering its half‐life it is not surprising that the effect of IVIg is not always maintained over a 3–4 week interval (Harbo et al, ; Pollard and Armati, ) . A recent study on patients who were referred to a neuromuscular clinic with the diagnosis of refractory CIDP showed that the most frequent intervention required for a response to IVIg was increasing the frequency of IVIg maintenance treatment from once every 4 weeks to once every 2 weeks (Kaplan and Brannagan, ) . Another report regarding patients who were considered to be IVIg unresponsive were in fact patients who showed a very short response to IVIg and were in need of IVIg maintenance treatment with a very short treatment interval (Debs et al, ) .…”

Section: High Peaks or High Troughs?mentioning

confidence: 99%

Maintenance IV immunoglobulin treatment in chronic inflammatory demyelinating polyradiculoneuropathy

Kuitwaard

Fokkink

Brusse

et al. 2017

J Peripheral Nervous Sys

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Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) patients treated with intravenous immunoglobulin (IVIg) usually start with a standard dosage of 2 g/kg bodyweight. Only a minority of patients has a sustained improvement, and most require ongoing maintenance treatment. Preferred IVIg regimens, however, vary considerably between doctors and at present it is unknown which is optimal. As there are also large differences in IVIg dosage and interval requirements between patients, optimal IVIg maintenance treatment of CIDP is even more complex. The lack of evidence-based guidelines on how IVIg maintenance treatment should be administered may potentially lead to under- or overtreatment of this expensive therapy. We provide an overview of published practical IVIg maintenance treatment regimens, IVIg maintenance schedules used in randomized controlled trials and one based upon our own long-term experience on how this treatment could be given in CIDP.

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Evaluation of patients with refractory chronic inflammatory demyelinating polyneuropathy

Cited by 43 publications

References 33 publications

Clinical factors, diagnostic delay, and residual deficits in chronic inflammatory demyelinating polyradiculoneuropathy

Clinical factors, diagnostic delay, and residual deficits in chronic inflammatory demyelinating polyradiculoneuropathy

Chronic Inflammatory Demyelinating Polyradiculoneuropathy

Maintenance IV immunoglobulin treatment in chronic inflammatory demyelinating polyradiculoneuropathy

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