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IV Edaravone ALS Survival Study

Intravenous edaravone treatment in ALS and survival: An exploratory, retrospective, administrative claims analysis

Year of Publication: 2022

Authors: Benjamin Rix Brooks, James D. Berry, Malgorzata Ciepielewska, ..., Melissa Hagan

Journal: eClinicalMedicine

Citation: eClinicalMedicine 2022;52:101590

Link: https://doi.org/10.1016/j.eclinm.2022.101590


Clinical Question

Does treatment with intravenous edaravone improve overall survival in patients with ALS compared to those not treated with IV edaravone in a real-world setting?

Bottom Line

IV edaravone treatment was associated with a 6-month longer median survival and 27% lower risk of death compared with non-IV edaravone-treated patients in a predominantly riluzole-treated US cohort, though adequately powered RCTs are needed to confirm this finding.

Major Points

  • This is the first study to show a statistically significant improved survival time associated with IV edaravone in ALS patients
  • Median overall survival was 29.5 months with edaravone vs 23.5 months without (6-month difference)
  • Risk of death was 27% lower in IV edaravone-treated cases (HR 0.73; 95% CI 0.59-0.91; p=0.005)
  • 65.4% of patients in both groups had history of riluzole prescription
  • Propensity score matching controlled for age, race, region, sex, insurance, cardiovascular disease, riluzole prescription, and disease severity surrogates
  • Sensitivity analysis using inverse probability weighting confirmed findings (HR 0.65; 95% CI 0.53-0.79; p<0.0001)
  • Median IV edaravone treatment duration was 8.6 months (IQR 3.5-14.8)
  • Pre-index disease duration was approximately 7 months for both groups

Design

Study Type: Retrospective, observational, propensity score-matched comparative effectiveness cohort study

Randomization:

Blinding: None (observational study)

Enrollment Period: 8 August 2017 to 31 March 2020

Follow-up Duration: Until 31 March 2021 (median edaravone treatment 8.6 months; observation up to 31 months post-index)

Countries: United States

Sample Size: 636

Analysis: Propensity score matching using nearest-neighbour method with caliper width 0.1 SD of logit scores; shared frailty Cox regression analysis; Kaplan-Meier survival curves with log-rank test; sensitivity analyses using bootstrap imputation and inverse probability weighting. Conducted using R version 4.0.3.


Inclusion Criteria

  • Age ≥18 years
  • Diagnosis of ALS (ICD-10-CM code G12.21 or ICD-9-CM code 335.20) on any claim in any inpatient or outpatient setting
  • Enrolled in Optum Clinformatics Data Mart
  • For cases: Claim for IV edaravone (HCPCS codes J1301, J3490, or C9493, or NDC 70510-2171-xx) between 8 August 2017 and 31 March 2020
  • For controls: No history of IV edaravone prescription

Exclusion Criteria

  • No ALS diagnosis code (ICD-10-CM G12.21 or ICD-9-CM 335.20)
  • Age <18 years

Arms

FieldIV edaravone-treated casesControl
InterventionIntravenous edaravone treatment (Radicava). Treatment may or may not have continued until death or censoring. Median treatment duration 8.6 months.No IV edaravone treatment. May or may not have received riluzole.
DurationVariable (up to 31 months observation)Variable (up to 31 months observation)

Outcomes

OutcomeTypeControlInterventionHR / OR / RRP-value
All-cause mortality confirmed via Optum De-identified CDM Database-Date of Death table (sourced from US Social Security Administration Death Master File and CMS claims)Primary196 deaths (61.6%); Median survival 23.5 months (95% CI 20.0-28.0)155 deaths (48.7%); Median survival 29.5 months (95% CI 25.4-35.9)0.730.005
Survival probability at 12 monthsSecondary0.704 (95% CI 0.654-0.759)0.837 (95% CI 0.797-0.880)
Survival probability at 18 monthsSecondary0.587 (95% CI 0.532-0.648)0.685 (95% CI 0.632-0.743)
Survival probability at 24 monthsSecondary0.490 (95% CI 0.434-0.554)0.591 (95% CI 0.533-0.655)
Survival probability at 30 monthsSecondary0.426 (95% CI 0.370-0.491)0.499 (95% CI 0.438-0.569)
Sensitivity analysis - Inverse probability weightingSecondaryReferenceHR 0.650.65<0.0001
All-cause mortality at 31 Mar 2021Adverse196/318 (61.6%)155/318 (48.7%)HR 0.73 (95% CI 0.59-0.91)0.005
Safety / adverse event reportingAdverseNot reported (retrospective administrative claims study - no safety data captured)

Subgroup Analysis

Not formally reported. Pre-specified matching variables included age, race, region, sex, insurance type, cardiovascular disease history, riluzole prescription, and disease severity surrogates (gastrostomy tube, artificial nutrition, noninvasive ventilation, all-cause hospitalisation). All standardised mean differences were <0.1 after matching, indicating adequate balance.


Criticisms

  • Retrospective observational design limits causal inference; an adequately powered RCT is needed to confirm findings
  • Administrative claims data subject to coding limitations and entry error
  • Study included only patients with commercial health coverage or Medicare Advantage plans, limiting generalisability
  • ALSFRS-R and FVC scores (clinical measures of disease severity) not available in claims database; surrogates used instead
  • Possibility of underdiagnosis of ALS may have led to selection bias
  • Adjustment limited to characteristics measurable from administrative claims; unmeasured confounding possible
  • Cases and controls may have differential censoring patterns
  • Index date for controls was the date edaravone became available (8 August 2017), not a treatment initiation date
  • Patients who continued edaravone may have been inherently healthier (selection bias)
  • Study population may appear healthier than total ALS population
  • No information on ALS phenotype or site of onset
  • Variable edaravone treatment duration among cases

Funding

Funded by Mitsubishi Tanabe Pharma America

Based on: IV Edaravone ALS Survival Study (eClinicalMedicine, 2022)

Authors: Benjamin Rix Brooks, James D. Berry, Malgorzata Ciepielewska, ..., Melissa Hagan

Citation: eClinicalMedicine 2022;52:101590

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