Is gene panel sequencing more efficient than clinical-based gene sequencing to diagnose autoinflammatory diseases? A randomized study.

Sanger sequencing genetic testing hereditary autoinflammatory diseases targeted next-generation sequencing

Journal

Clinical and experimental immunology
ISSN: 1365-2249
Titre abrégé: Clin Exp Immunol
Pays: England
ID NLM: 0057202

Informations de publication

Date de publication:
01 2021
Historique:
received: 17 04 2020
revised: 31 07 2020
accepted: 12 08 2020
pubmed: 11 9 2020
medline: 27 7 2021
entrez: 10 9 2020
Statut: ppublish

Résumé

The aim of this study was to compare the effectiveness of the gene-panel next-generation sequencing (NGS) strategy versus the clinical-based gene Sanger sequencing for the genetic diagnosis of autoinflammatory diseases (AIDs). Secondary goals were to describe the gene and mutation distribution in AID patients and to evaluate the impact of the genetic report on the patient's medical care and treatment. Patients with AID symptoms were enrolled prospectively and randomized to two arms, NGS (n = 99) (32-55 genes) and Sanger sequencing (n = 197) (one to four genes). Genotypes were classified as 'consistent/confirmatory', 'uncertain significance' or 'non-contributory'. The proportion of patients with pathogenic genotypes concordant with the AID phenotype (consistent/confirmatory) was significantly higher with NGS than Sanger sequencing [10 of 99 (10·1%) versus eight of 197 (4·1%)]. MEFV, ADA2 and MVK were the most represented genes with a consistent/confirmed genotype, whereas MEFV, NLRP3, NOD2 and TNFRSF1A were found in the 'uncertain significance' genotypes. Six months after the genetic report was sent, 54 of 128 (42·2%) patients had received effective treatment for their symptoms; 13 of 128 (10·2%) had started treatment after the genetic study. For 59 of 128 (46%) patients, the results had an impact on their overall care, independent of sequencing group and diagnostic conclusion. Targeted NGS improved the diagnosis and global care of patients with AIDs.

Identifiants

pubmed: 32909274
doi: 10.1111/cei.13511
pmc: PMC7744495
doi:

Banques de données

ClinicalTrials.gov
['NCT02976948']

Types de publication

Journal Article Randomized Controlled Trial Research Support, Non-U.S. Gov't

Langues

eng

Sous-ensembles de citation

IM

Pagination

105-114

Informations de copyright

© 2020 British Society for Immunology.

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Auteurs

M Rama (M)

Department of Medical Genetics, Rare Diseases and Personalized Medicine, Rare and Autoinflammatory Diseases Unit, CHU Montpellier, Univ Montpellier, Montpellier, France.

T Mura (T)

Medical Information Department, Montpellier University Hospital, Montpellier, France.

I Kone-Paut (I)

Department of Paediatric Rheumatology, CEREMAIA, Bicêtre Hospital, AP-HP, University of Paris Sud Saclay, AP-HP, Le Kremlin-Bicêtre, France.

G Boursier (G)

Department of Medical Genetics, Rare Diseases and Personalized Medicine, Rare and Autoinflammatory Diseases Unit, CHU Montpellier, Univ Montpellier, Montpellier, France.

S Aouinti (S)

Department of Paediatric Rheumatology, CEREMAIA, Bicêtre Hospital, AP-HP, University of Paris Sud Saclay, AP-HP, Le Kremlin-Bicêtre, France.

I Touitou (I)

Department of Medical Genetics, Rare Diseases and Personalized Medicine, Rare and Autoinflammatory Diseases Unit, CHU Montpellier, Univ Montpellier, Montpellier, France.
IRMB, University of Montpellier, INSERM, CEREMAIA CHU Montpellier, Montpellier, France.

G Sarrabay (G)

Department of Medical Genetics, Rare Diseases and Personalized Medicine, Rare and Autoinflammatory Diseases Unit, CHU Montpellier, Univ Montpellier, Montpellier, France.
IRMB, University of Montpellier, INSERM, CEREMAIA CHU Montpellier, Montpellier, France.

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Classifications MeSH