Evidence map›Paper›PMID 37752496›Full record

ArticlePediatric rheumatology online journal2023

Treat-to-target strategies for the management of familial Mediterranean Fever in children.

Lisa Ehlers, Elisabeth Rolfes, Mareike Lieber, Dominik Müller, Elke Lainka, Faekah Gohar, Günter Klaus, Hermann Girschick, Jana Hörstermann, Jasmin Kümmerle-Deschner and 15 more

Open access · goldAbstract read
In one paragraph

Article in Pediatric rheumatology online journal, 2023. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 10 papers.

0numbers the graph read from it
0cells of the map it votes in
10citing papers in PubMed
2.2field-weighted citation impact, top 12% of its field
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

10 citing papers in PubMed, 14 citations in OpenAlex.

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4 · The record

Corrections and comments

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

25 authors at 13 institutions in 4 countries.

Lisa EhlersDepartment of Paediatric Pulmonology, Immunology and Critical Care Medicine, Charité - Universitätsmedizin Berlin, corporate member of Freie Universität Berlin and Humboldt-Universität zu Berlin, Berlin, Germany.
Elisabeth RolfesDepartment of Paediatric Pulmonology, Immunology and Critical Care Medicine, Charité - Universitätsmedizin Berlin, corporate member of Freie Universität Berlin and Humboldt-Universität zu Berlin, Berlin, Germany.
Mareike LieberDepartment of Paediatric Pulmonology, Immunology and Critical Care Medicine, Charité - Universitätsmedizin Berlin, corporate member of Freie Universität Berlin and Humboldt-Universität zu Berlin, Berlin, Germany.
Dominik MüllerDepartment of Paediatrics, Division of Gastroenterology, Nephrology and Metabolic Diseases, Charité - Universitätsmedizin Berlin, corporate member of Freie Universität Berlin and Humboldt-Universität zu Berlin, Berlin, Germany.
Elke LainkaDepartment of Paediatrics II, University Hospital Essen, Children's Hospital, Essen, Germany.
Faekah GoharClinic of Paediatric and Adolescent Rheumatology, St. Josef-Stift Sendenhorst, Northwest German Center for Rheumatology, Sendenhorst, Germany.
Günter KlausKfH Center of Paediatric Nephrology, Department of Paediatric Nephrology, Marburg, Germany.
Hermann GirschickVivantes Klinikum Friedrichshain, Children's Hospital, Berlin, Germany.
Jana HörstermannDeutsches Rheuma-Forschungszentrum (DRFZ), An Institute of the Leibniz Association, Berlin, Germany.
Jasmin Kümmerle-DeschnerAutoinflammation Reference Center Tübingen (arcT), Division of Paediatric Rheumatology, Department of Paediatrics, University Hospital Tübingen, Tübingen, Germany.
Jürgen BrunnerDepartment of Paediatrics, Medical University Innsbruck, Danube Private University, Innsbruck, Krems, Austria.
Katharina Palm-BedenClinic of Paediatric and Adolescent Rheumatology, St. Josef-Stift Sendenhorst, Northwest German Center for Rheumatology, Sendenhorst, Germany.
Klaus TenbrockDepartment of Paediatric Pneumology, Allergology and Immunology, RWTH Aachen, Aachen, Germany.
Lusine von WrangelPatient representative, Berlin, Germany.
Maria FaßhauerImmunoDeficiencyCenter Leipzig (IDCL), Hospital St. Georg GmbH Leipzig, Academic Teaching Hospital of the University of Leipzig, Leipzig, Germany.
Norbert BlankDepartment of Hematology, Oncology and Rheumatology, Internal Medicine V, University Hospital of Heidelberg, Heidelberg, Germany.
Ralf TrauzeddelDepartment of Paediatrics, Helios Klinikum Berlin-Buch, Berlin, Germany.
Anne Sae Lim von StuckradDepartment of Paediatric Pulmonology, Immunology and Critical Care Medicine, Charité - Universitätsmedizin Berlin, corporate member of Freie Universität Berlin and Humboldt-Universität zu Berlin, Berlin, Germany.
Sonja HigginsPaediatric medical practice Hürthpark, Hürth, Germany.
Tatjana WelzelAutoinflammation Reference Center Tübingen (arcT), Division of Paediatric Rheumatology, Department of Paediatrics, University Hospital Tübingen, Tübingen, Germany.
Thomas LutzCenter for Rheumatology, Paediatric Rheumatology, Heidelberg, Germany.
Véronique HentgenDepartment of Paediatrics, National Reference Center for Auto-inflammatory Diseases and Amyloidosis, CEREMAIA, Versailles Hospital, Versailles, France.
Dirk FoellDepartment of Paediatric Rheumatology and Immunology, University Hospital Münster, Münster, Germany.
Helmut WittkowskiDepartment of Paediatric Rheumatology and Immunology, University Hospital Münster, Münster, Germany.
Tilmann KallinichDepartment of Paediatric Pulmonology, Immunology and Critical Care Medicine, Charité - Universitätsmedizin Berlin, corporate member of Freie Universität Berlin and Humboldt-Universität zu Berlin, Berlin, Germany. tilmann.kallinich@charite.de.ORCID http://orcid.org/0000-0003-2404-9397
Humboldt-Universität zu Berlin · DEUniversity Hospital Münster · DECentre de Référence des Maladies Autoinflammatoires et des Amyloses · FRDanube Private University · ATEssen University Hospital · DEGerman Center for Pediatric and Adolescent Rheumatology · DEHeidelberg University · DEHelios Hospital Berlin-Buch · DEKlinikum im Friedrichshain · DEKlinikum St. Georg · DELeibniz Association · DERWTH Aachen University · DEUniversity Children’s Hospital Basel · CH

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

backgroundThe objective of this initiative was to develop a treat-to-target (T2T) approach for the management of patients with Familial Mediterranean Fever (FMF), including the definition of a complex treatment target, and establish strategies that improve patient care and long-term outcome.

methodsAn initial set of statements as well as a flow chart visualising the proposed concept was developed. To adapt the preliminary statements to the current state of knowledge, a systematic literature search was performed and the modified statements were subject to a Delphi approach. To ensure the applicability of the statements in daily practice, an online survey was conducted among paediatric rheumatologists in Germany. In addition, data from the national AID-NET registry were analysed with respect to therapeutic response.

resultsThis T2T initiative yielded a total of 26 statements guiding FMF management with respect to diagnosis, treatment targets, treatment strategies and monitoring. The online survey identified cut-off values for inflammatory markers indicating treatment intensification and appropriate measures in case of colchicine intolerance or non-adherence. The analysis of data derived from the national AID-NET showed that colchicine therapy was successfully terminated in 61% of patients (27 out of 44) with heterozygous MEFV mutations. Multidimensional treatment targets incorporating objective and subjective reported outcome measures were developed. These provide the basis for stratifying patients into the following treatment paths: continue colchicine, persisting attacks / inflammation, colchicine intolerance, persisting arthritis, colchicine reduction and adjustment/reduction of biologics.

conclusionsThe proposed consensus treatment plan for the management of FMF incorporates multidimensional targets allowing transparent treatment decisions, which will promote personalised disease management and increase adherence to therapy.

Indexed as

ArthritisBiological ProductsFamilial Mediterranean FeverChildColchicineConsensusHumansPractice Guidelines as TopicPyrinBiological ProductsColchicineMEFV protein, humanPyrinChildrenColchicineColchicine resistanceDisease activityFamilial Mediterranean Feverinterleukin-1 antagonistsMedication adherenceTreat-to-target

Identifiers

PMID37752496
PMCPMC10521475
OpenAlexW4387053991

What OpenQuestion holds

Textmetadata
LicenceCC BY
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Registered trials

None linked

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 OpenQuestion graph.