Evidence mapPaperPMID 38988097Full record

ArticleEuropean journal of neurology2024

Effectiveness of patisiran after switching from tafamidis for the treatment of hereditary transthyretin-mediated amyloidosis with polyneuropathy.

Celine Labeyrie, Madeline Merkel, Sakshi Sethi, Lyuba Popadic, Hongbo Yang, Marianne T Sweetser, Hollis Lin, David Adams

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Article in European journal of neurology, 2024. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 2 papers.

0numbers the graph read from it
0cells of the map it votes in
2citing papers in PubMed
field-weighted citation impact
1 · What the graph read from it

What it found

Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.

The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.

2 · The registry

The trial behind it

Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.

Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.

3 · Its place in the literature

Who cites it

2 citing papers in PubMed.

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4 · The record

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PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.

5 · Who and what money

Authors and funding

8 authors.

Celine LabeyrieNeurology Department, Assistance Publique-Hopitaux de Paris (AP-HP), CERAMIC, Centre Hospitalier Universitaire (CHU) Bicêtre, INSERM U1195, Université Paris-Saclay, Le Kremlin-Bicêtre, France.ORCID 0000-0001-9765-709X
Madeline MerkelAlnylam Pharmaceuticals, Cambridge, Massachusetts, USA.
Sakshi SethiAnalysis Group, Boston, Massachusetts, USA.
Lyuba PopadicAnalysis Group, Boston, Massachusetts, USA.
Hongbo YangAnalysis Group, Boston, Massachusetts, USA.ORCID 0000-0003-2741-8418
Marianne T SweetserAlnylam Pharmaceuticals, Cambridge, Massachusetts, USA.
Hollis LinAlnylam Pharmaceuticals, Cambridge, Massachusetts, USA.
David AdamsNeurology Department, Assistance Publique-Hopitaux de Paris (AP-HP), CERAMIC, Centre Hospitalier Universitaire (CHU) Bicêtre, INSERM U1195, Université Paris-Saclay, Le Kremlin-Bicêtre, France.ORCID 0000-0002-8722-4108

Funding

Alnylam Pharmaceuticals
6 · The paper itself

Abstract

background and purposeHereditary transthyretin-mediated amyloidosis with polyneuropathy (ATTRv-PN [v for variant]) is a rare, progressive disease associated with multisystemic impairments. This study assessed the real-world outcomes of patients with ATTRv-PN who switched from tafamidis to patisiran, as well as the reasons for the treatment switch.

methodsThis was a retrospective chart review study at a large expert referral center. Data were extracted from medical charts of patients with ATTRv-PN who switched from tafamidis to patisiran on or before 30 August 2019. Data elements included demographic and clinical characteristics, rationale for switch, and disease measures evaluated from tafamidis initiation through the 12-month patisiran treatment period.

resultsAmong the 24 patients with ATTRv-PN included in the study, 50.0% had a V30M variant, and the mean (SD) age was 67.3 (8.0) years. During tafamidis treatment (mean [SD] = 30.1 [17.5] months) before switching to patisiran, patients worsened across multiple polyneuropathy measures, including walking ability, Neuropathy Impairment Score, and autonomic function. Neuropathic disease progression on tafamidis was the principal reason for switching to patisiran. After 12 months on patisiran (mean [SD] = 11.7 [1.4] months), patients experienced attenuated disease progression or improvement in the aforementioned measures of polyneuropathy.

conclusionsSwitching from tafamidis to patisiran attenuated the rate of functional decline, and most patients experienced stabilization or improvement of at least one polyneuropathy measure within 12 months of patisiran treatment. Timely switch from tafamidis to patisiran can be beneficial to avoid rapid disease progression in patients with ATTRv-PN.

Indexed as

Amyloid Neuropathies, FamilialBenzoxazolesPolyneuropathiesAgedDrug SubstitutionFemaleHumansMaleMiddle AgedRetrospective StudiesRNA, Small InterferingTreatment OutcomeBenzoxazolespatisiranRNA, Small Interferingtafamidisfamilial amyloidosispatisiranpolyneuropathytafamidistransthyretin

Identifiers

PMID38988097
PMCPMC11295162

What Socratic holds

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Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the Socratic graph.