Pharmacological treatment other than corticosteroids, intravenous immunoglobulin and plasma exchange for Guillain-Barre syndrome.
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ABSTRACT:
SUBMITTER: Doets AY
PROVIDER: S-EPMC6984651 | biostudies-literature | 2020 Jan
REPOSITORIES: biostudies-literature
Publications
Pharmacological treatment other than corticosteroids, intravenous immunoglobulin and plasma exchange for Guillain-Barré syndrome.
Doets Alex Y AY Hughes Richard Ac RA Brassington Ruth R Hadden Robert Dm RD Pritchard Jane J
The Cochrane database of systematic reviews 20200125
<h4>Background</h4>Plasma exchange and intravenous immunoglobulin, but not corticosteroids, are beneficial in Guillain-Barré syndrome (GBS). The efficacy of other pharmacological agents is unknown. This review was first published in 2011 and previously updated in 2013, and 2016.<h4>Objectives</h4>To assess the effects of pharmacological agents other than plasma exchange, intravenous immunoglobulin and corticosteroids for GBS.<h4>Search methods</h4>On 28 October 2019, we searched the Cochrane Neu ...[more]
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Project description:BackgroundPlasma exchange and intravenous immunoglobulin, but not corticosteroids, are beneficial in Guillain-Barré syndrome (GBS). The efficacy of other pharmacological agents is unknown. This review was first published in 2011 and updated in 2013 and 2016.ObjectivesTo assess the effects of pharmacological agents other than plasma exchange, intravenous immunoglobulin and corticosteroids for GBS.Search methodsOn 18 January 2016, we searched the Cochrane Neuromuscular Specialised Register, Cochrane Central Register of Controlled Trials, MEDLINE, and Embase for treatments for GBS. We also searched clinical trials registries.Selection criteriaWe included all randomised controlled trials (RCTs) or quasi-RCTs of acute GBS (within four weeks from onset) of all types and degrees of severity, and in individuals of all ages. We discarded trials that investigated only corticosteroids, intravenous immunoglobulin or plasma exchange. We included other pharmacological treatments or combinations of treatments compared with no treatment, placebo or another treatment. We also identified a number of non-randomised studies during the search, the results of which we considered in the Discussion.Data collection and analysisWe followed standard Cochrane methodology.Main resultsWe identified no new trials during this update of the review. In previous versions of this review we identified only very low quality evidence for four different interventions published in four studies. Each study had a high risk of bias in at least one respect. One RCT with 19 participants comparing interferon beta-1a and placebo showed no clinically important difference in any outcome between groups. Another with 10 participants comparing brain-derived neurotrophic factor and placebo showed no clinically important difference in any outcome between groups. A third with 37 participants comparing cerebrospinal fluid filtration and plasma exchange also showed no clinically important difference in any outcome between groups. In a fourth with 43 participants, the risk ratio for an improvement by one or more disability grade after eight weeks was greater with the Chinese herbal medicine tripterygium polyglycoside than with corticosteroids (risk ratio 1.47; 95% confidence interval 1.02 to 2.11); other outcomes in this trial showed no difference. Serious adverse events were uncommon with each of these treatments and in the control groups.Authors' conclusionsThe quality of the evidence was very low. Three small RCTs, comparing interferon beta-1a or brain-derived neurotrophic factor with placebo, and cerebrospinal fluid filtration with plasma exchange, showed no significant benefit or harm for any of the interventions. A fourth small trial showed that the Chinese herbal medicine, tripterygium polyglycoside, hastened recovery in people with GBS to a greater extent than corticosteroids, but this result needs confirmation. We were unable to draw any useful conclusions from the few observational studies we identified.
| S-EPMC6464710 | biostudies-literature
Project description:IntroductionRespiratory failure is a life threatening complication of Guillain Barré syndrome (GBS). There is no consensus on the specific treatment for this subset of children with GBS.MethodsThis was a prospective randomized study to compare the outcome of intravenous immunoglobulin (IVIG) and plasma exchange (PE) treatment in children with GBS requiring mechanical ventilation. Forty-one children with GBS requiring endotracheal mechanical ventilation (MV) within 14 days from disease onset were included. The ages of the children ranged from 49 to 143 months.Randomly, 20 children received a five-day course of IVIG (0.4 g/kg/day) and 21 children received a five-day course of one volume PE daily. Lumbar puncture (LP) was performed in 36 patients (18 in each group).ResultsBoth groups had comparable age (p = 0.764), weight (p = 0.764), duration of illness prior to MV (p = 0.854), preceding diarrhea (p = 0.751), cranial nerve involvement (p = 0.756), muscle power using Medical Research Council (MRC) sum score (p = 0.266) and cerebrospinal fluid (CSF) protein (p = 0.606).Children in the PE group had a shorter period of MV (median 11 days, IQR 11.0 to 13.0) compared to IVIG group (median 13 days, IQR 11.3 to 14.5) with p = 0.037.Those in the PE group had a tendency for a shorter Pediatric Intensive Care Unit (PICU) stay (p = 0.094).A total of 20/21 (95.2%) and 18/20 (90%) children in the PE and IVIG groups respectively could walk unaided within four weeks after PICU discharge (p = 0.606).There was a negative correlation between CSF protein and duration of mechanical ventilation in the PE group (p = 0.037), but not in the IVIG group (p = 0.132).ConclusionsIn children with GBS requiring MV, PE is superior to IVIG regarding the duration of MV but not PICU stay or the short term neurological outcome.The negative correlation between CSF protein values and duration of MV in PE group requires further evaluation of its clinical usefulness.Trial registrationClinicaltrials.gov Identifier NCT01306578.
| S-EPMC3387601 | biostudies-literature
Project description:Background and objectiveIntravenous immunoglobulin (IVIg) at a standard dosage is the treatment of choice for Guillain-Barré syndrome. The pharmacokinetics, however, is highly variable between patients, and a rapid clearance of IVIg is associated with poor recovery. We aimed to develop a model to predict the pharmacokinetics of a standard 5-day IVIg course (0.4 g/kg/day) in patients with Guillain-Barré syndrome.MethodsNon-linear mixed-effects modelling software (NONMEM®) was used to construct a pharmacokinetic model based on a model-building cohort of 177 patients with Guillain-Barré syndrome, with a total of 589 sequential serum samples tested for total immunoglobulin G (IgG) levels, and evaluated on an independent validation cohort that consisted of 177 patients with Guillain-Barré syndrome with 689 sequential serum samples.ResultsThe final two-compartment model accurately described the daily increment in serum IgG levels during a standard IVIg course; the initial rapid fall and then a gradual decline to steady-state levels thereafter. The covariates that increased IgG clearance were a more severe disease (as indicated by the Guillain-Barré syndrome disability score) and concomitant methylprednisolone treatment. When the current dosing regimen was simulated, the percentage of patients who reached a target ∆IgG > 7.3 g/L at 2 weeks decreased from 74% in mildly affected patients to only 33% in the most severely affected and mechanically ventilated patients (Guillain-Barré syndrome disability score of 5).ConclusionsThis is the first population-pharmacokinetic model for standard IVIg treatment in Guillain-Barré syndrome. The model provides a new tool to predict the pharmacokinetics of alternative regimens of IVIg in Guillain-Barré syndrome to design future trials and personalise treatment.
| S-EPMC9439991 | biostudies-literature
Project description:BACKGROUND: High doses of pooled polyclonal IgG are commonly used to treat numerous autoimmune diseases. Their mode of action nevertheless remains only partially explained. At the same time, until now, no early biological marker has been able to predict their efficacy. METHODOLOGY/PRINCIPAL FINDINGS: In a first pilot retrospective analysis, we reviewed white blood cell counts and blood smears in consecutive patients with autoimmune disease (n = 202) and non-autoimmune disease (n = 104). Autoimmune patients received either intravenous immunoglobulin (IVIg, n = 103), plasma exchange (n = 78) or no specific treatment (n = 21). We then prospectively monitored consecutive autoimmune patients with IVIg injection (n = 67), or without any specific treatment (n = 10) using the same routine laboratory tests, as well as flow cytometry. Both retrospective and prospective analyses identified large plasma-cell mobilization exclusively in IVIg-treated autoimmune patients 7 days after initiation of treatment. The majority of IVIg-mobilized plasma cells were immature HLA-DR(high)/CD138(low)/CXCR4(low) plasma cells expressing intracellular immunoglobulin G which were neither IVIg- nor human IgG-specific. Importantly, we found a strong negative correlation between the absolute number of IVIg-mobilized plasma cells and time to improve neurological function in both retrospective and prospective studies of Guillain-Barré syndrome (GBS), (r = -0.52, p = 0.0031, n = 30, r = -0.47, p = 0.0028, n = 40, respectively). CONCLUSIONS/SIGNIFICANCE: IVIg promotes immature plasma-cell mobilization in patients with GBS, chronic inflammatory demyelinating polyneuropathy, myasthenia gravis and inflammatory myopathy. Prominent day 7 plasma-cell mobilization is a favourable prognostic marker in patients with GBS receiving IVIg treatment.
| S-EPMC2362102 | biostudies-literature
Project description:IntroductionGuillain-Barré Syndrome (GBS) is an acute, immune-mediated polyneuropathy that often leads to severe weakness. Intravenous Immunoglobulin (IVIG) is a proven effective treatment for GBS (class 1 evidence). The clinical course of GBS in individual patients is highly variable and difficult to predict.MethodsIt is a retrospective observational study of 10 patients diagnosed with GBS by using nerve conduction studies and lumbar puncture.ResultsFifty percent of the patients were under 40 years old, and ninety percent were male; all but one were treated with IVIG; and forty percent of the total mEGOS obtained was less than 5. The average predicted probability of being unable to walk unaided after 4 weeks was 47.7%, the average predicted probability of being unable to walk unaided after 3 months was 17%, and the average predicted probability of being unable to walk unaided after 6 months was 8.05%.ConclusionsPatients presenting with acute ascending weakness should be identified early, and early IVIG treatment for GBS improves disability as measured by The Modified Erasmus GBS Outcome Scale (mEGOS).
| S-EPMC9758282 | biostudies-literature
Project description:Corticosteroids have been proved to be ineffective for Guillain-Barré syndrome, but the mechanism remains unknown. In a rabbit model of axonal Guillain-Barré syndrome, treatment with corticosteroids significantly reduced macrophage infiltration in the spinal ventral roots and the survival rate as well as clinical improvement. On 30(th) day after onset, there was significantly higher frequency of axonal degeneration in the corticosteroids-treated rabbits than saline-treated rabbits. Corticosteroids may reduce the scavengers that play a crucial role for nerve regeneration, thus delay the recovery of this disease.
| S-EPMC4565078 | biostudies-other
Project description:Guillain-Barré syndrome (GBS) is an acute autoimmune polyradiculoneuropathy affecting 1-2 subjects per 100,000 every year worldwide. It causes, in its classic form, symmetric weakness in the proximal and distal limb muscles with common involvement of the cranial nerves, particularly facial weakness. Respiratory function is compromised in a case in four. Randomised controlled trials have demonstrated the benefit of therapeutic plasma exchange in hastening time to recovery. Intravenous immunoglobulin was subsequently shown to be as efficacious as plasma exchange in adult subjects. In children, few trials have shown the benefit of intravenous immunoglobulin versus supportive care. Pharmacokinetic studies suggested a relationship between increase in immunoglobulin G level post-infusion and outcome, implying administration of larger doses may be beneficial in subjects with poor prognosis. However, a subsequent trial of a second dose of immunoglobulin in such subjects failed to show improved outcome, while demonstrating a higher risk of thromboembolic side-effects. Monoclonal antibody therapy has more recently been investigated for GBS, after multiple studies in animal models, with different agents and variable postulated mechanisms of action. Eculizumab, a humanised monoclonal antibody against the complement protein C5, was tested in in two randomised, double-blind, placebo-controlled phase 2 trials. Neither showed benefit versus immunoglobulins alone on disability level at 4 weeks, although one study importantly suggested possible, clinically highly relevant, late effects on normalising function. A phase 3 trial is in progress. Preliminary results of a placebo-controlled ongoing study of ANX005, a humanised recombinant antibody against C1q inhibiting the complement cascade, have been promising.
| S-EPMC9159039 | biostudies-literature
Project description:IntroductionSevere Guillain-Barré syndrome (GBS) patients may not show improvement after a single course of intravenous immunoglobulin (IVIg) therapy. Current treatment options include either a second course of IVIg or therapeutic plasma exchange (TPE). This systematic review aims to evaluate the current literature on the use of a second course of IVIg or TPE in patients who fail to show clinical improvement after the first IVIg course.MethodsWe searched PubMed, Embase and Medline databases up until 26 October 2023. Studies that evaluated adult patients with confirmed GBS who have failed one full course of IVIg and subsequently received either repeat IVIg or TPE were included. Risk of bias was performed using study-specific checklists. A narrative synthesis of results is presented.ResultsA total of 37 articles were identified (1 randomised controlled trial (RCT), 3 observational and 33 case reports/series), consisting of 422 patients in total. 12 studies evaluated repeat IVIg and 24 studies evaluated TPE after IVIg. There was no superiority of a repeat course of IVIg or TPE in all clinical outcome measures.ConclusionsThe evidence suggests with a low degree of certainty that there is no beneficial effect of further IVIg in unresponsive GBS. The quality of evidence regarding TPE after IVIg is insufficient to suggest any efficacy due to a lack of RCTs. We recommend standardised case reporting with consideration for a multinational case registry and RCTs to determine the efficacy of TPE after initial IVIg unresponsiveness.
| S-EPMC11822392 | biostudies-literature
Project description:BackgroundIn Bangladesh, most patients with Guillain-Barré syndrome (GBS) cannot afford standard treatment with intravenous immunoglobulin (IVIG) or a standard plasma exchange (PE) course, which partly explains the high rate of mortality and residual disability associated with GBS in this country. Small volume plasma exchange (SVPE) is an affordable and potentially effective alternative form of plasma exchange. SVPE is the repeated removal of small volumes of supernatant plasma over several days via sedimentation of patient whole blood. The aim of this study is to define the clinical feasibility and safety of SVPE in patients with GBS in resource poor settings.MethodsA total of 20 adult patients with GBS will be enrolled for SVPE at a single center in Bangladesh. Six daily sessions of whole blood sedimentation and plasma removal will be performed in all patients with GBS with a target to remove an overall volume of at least 8 liters (L) of plasma over a total of 8 days. Serious adverse events (SAE) are defined as the number of patients developing severe sepsis associated with the central venous catheter or deep venous thrombosis in the limb where the catheter is placed for SVPE. Based upon a predictive success rate of 75%, the SVPE procedure will be considered safe if less than 5 of 20 SVPE-treated GBS patients have a SAE. The procedure will be considered feasible if 8 L of plasma can be removed in at least 15 of 20 patients with GBS who receive SVPE. In addition, detailed clinical and neurological outcome assessments will be performed until discharge of the patient from the hospital and up to 4 weeks after study entry.DiscussionThis is the first clinical study to evaluate the feasibility and safety of SVPE as a potential alternative low-cost treatment for the patients with GBS in resource poor settings.Trial registrationClinicaltrials.gov NCT02780570.
| S-EPMC5622586 | biostudies-literature
Project description:Guillain-Barre Syndrome (GBS) is an inflammatory disease of the peripheral nervous system. Given that plasma metabolic profiles in GBS patients have never been explored, plasma samples of 38 GBS patients, 22 multiple sclerosis (MS) patients, and 40 healthy controls were analyzed by using untargeted and targeted metabolomics analysis. The untargeted analysis showed that levels of a set of plasma lipid metabolites were significantly decreased in GBS patients compared to the controls. Furthermore, the targeted analysis demonstrated that levels of 41 metabolites in GBS patients were significantly changed compared to either the controls or MS patients. A further metabolic analysis showed that 12 of 41 metabolites were significantly lower in classical GBS patients compared to Miller-Fisher syndrome. Among them, each of PCae C34:0, PCae C42:2, PCae C42:3, and SM C24:0 was inversely correlated with Hughes functional grading scale of GBS patients at both nadir and discharge. Receiver operating characteristic curve analysis of combination of three metabolites (PCaa C42:2, PCae C36:0 and SM C24:0) showed a good discrimination between the GBS and the controls (area under curve = 0.86). This study has demonstrated disruption of lipid metabolites in GBS may be potential biomarkers to indicate disease severity and prognosis of GBS.
| S-EPMC5557802 | biostudies-literature