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

Kuitwaard, Krista; Hahn, Angelika F.; Vermeulen, M.; Venance, Shannon L.; Doorn, Pieter A. van

doi:10.1136/jnnp-2014-309042

Cited by 50 publications

(63 citation statements)

References 48 publications

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“…Diagnostic consensus was reached in the 112 remaining patients. Fifteen of these patients have been described previously …”

Section: Resultsmentioning

confidence: 99%

“…[15][16][17][18] If one treatment fails, one of the other treatment types is often effective. 19 The treatment of CIDP is complicated by the variable responsiveness of individual patients. Although dosing algorithms for IVIg treatment are suggested, there is no gold standard available on how to select treatment type, regimen, and how to monitor maintenance treatments.…”

Section: Introductionmentioning

confidence: 99%

See 1 more Smart Citation

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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“…Diagnostic consensus was reached in the 112 remaining patients. Fifteen of these patients have been described previously …”

Section: Resultsmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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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“…Immunotherapies that have been shown to be effective in the treatment of CIDP include glucocorticoids, intravenous immunoglobulin (IVIG), subcutaneous immunoglobulin, and plasma exchange . Intravenous immunoglobulin is the first‐line treatment in most patients . Steroids, administered in pulsed or daily doses, are also used as first‐line treatment, but their associated adverse effects make long‐term treatment difficult .…”

Section: Introductionmentioning

confidence: 99%

Rituximab in refractory chronic inflammatory demyelinating polyneuropathy

et al. 2020

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Introduction: Chronic inflammatory demyelinating polyneuropathy (CIDP) is a disorder in which early effective treatment is important to minimize disability from axonal degeneration. It has been suggested that some patients with CIDP may benefit from rituximab therapy, but there is no definitive evidence for this. Methods: Baseline and post-rituximab-therapy neuromuscular Medical Research Council (MRC) sum scores, Inflammatory Neuropathy Cause and Treatment (INCAT) disability score, and functional status were assessed in 11 patients with refactory CIDP.Results: The MRC sum score, INCAT disability score, and functional status improved in all patients after rituximab therapy. Discussion:Our study provides evidence of the efficacy of rituximab therapy in at least some patients with CIDP. A placebo-controlled study to assess the effectiveness of rituximab therapy in CIDP with and without nodal antibodies is required to identify disease markers that predict responsiveness. K E Y W O R D S

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“…In our study, most patients needed long‐term IVIg maintenance therapy and IVIg withdrawal attempts were successful in only 21% of patients, which is in line with other studies in asymmetric CIDP variants . In a large retrospective study, including typical and atypical CIDP, 40% of IVIg responsive patients were in remission after a mean follow up of 5 years, suggesting that patients with an asymmetric CIDP variant might be more dependent on long‐term IVIg maintenance treatment than CIDP patients in general.…”

Section: Discussionmentioning

confidence: 99%

“…of IVIg responsive patients were in remission after a mean follow up of 5 years, 30 suggesting that patients with an asymmetric CIDP (7) To Erb OS c 78% (25) 13% (4) 9% (3) Legs d 78% (25) 19% (6) 3% (1) Radial and musculocutaneous nerve 78% (25) 22% (7)…”

Section: Long-term Follow-upmentioning

confidence: 99%

Diagnosis and treatment response in the asymmetric variant of chronic inflammatory demyelinating polyneuropathy

Lucke

Kooi

Schaik

et al. 2019

J Peripheral Nervous Sys

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The objectives were to (a) assess the diagnostic value of testing clinically affected and unaffected limbs with nerve conduction studies (NCS) in patients with the asymmetric chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) variant and to define the most useful strategy for diagnosis, and (b) describe treatment response and long‐term outcome. We performed a retrospective study and included patients with a multifocal distribution of symptoms and signs, who met the probable or definite EFNS/PNS diagnostic categories for CIDP. We included 34 patients and 32 NCS datasets were available. Of these 32 patients, 25 (78%) met the electrodiagnostic criteria for definite or probable CIDP and seven (22%) for possible CIDP. Patients fulfilling the possible electrodiagnostic criteria and a supportive criterion were considered as probable CIDP. NCS of the clinically affected forearm and leg led to a probable or definite diagnosis in 13 patients (41%). Measuring both arms up to Erb's point led to a probable or definite diagnosis in 25 patients (78%), after which NCS of both legs did not contribute to additional probable or definite diagnoses. In total, 30% of patients treated with dexamethasone and 94% of patients treated with intravenous immunoglobulins (IVIg) responded. IVIg withdrawal attempts were successful in 21% of patients. After measuring the clinically affected arm up to Erb's point, NCS of the unaffected arm to Erb's point has the highest additional diagnostic yield in patients with asymmetric CIDP. Patients seem to respond better to IVIg than to corticosteroids and long‐term treatment is often required, although IVIg withdrawal was successful in 21%.

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

Abstract: IVIg is a very effective first-line treatment. Patients with CIDP presenting with pain or a difference in weakness between arms and legs are less likely to respond to IVIg.

Cited by 50 publications

References 48 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

Rituximab in refractory chronic inflammatory demyelinating polyneuropathy

Diagnosis and treatment response in the asymmetric variant of chronic inflammatory demyelinating polyneuropathy

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