Evidence map›Paper›PMID 34527082›Full record

ArticleTherapeutic advances in musculoskeletal disease2021

Anakinra and canakinumab for patients with R92Q-associated autoinflammatory syndrome: a multicenter observational study from the AIDA Network.

Carla Gaggiano, Donato Rigante, José Hernández-Rodríguez, Antonio Vitale, Maria Tarsia, Alessandra Soriano, Giuseppe Lopalco, Florenzo Iannone, Masen Abdel Jaber, Roberto Giacomelli and 18 more

Registry-linked trialOpen access · goldAbstract read
PubMed Full text at PMC Publisher
In one paragraph

Article in Therapeutic advances in musculoskeletal disease, 2021. The graph could read no effect estimate from its abstract, so it casts no vote on the map. It is linked to trial NCT05200715 (Development of an International Multicenter Registry of Patients With Monogenic and Polygenic Autoinflammatory Diseases Aimed at Clinical and Therapeutical Data Collection and Analysis), which is not on this map. Cited by 2 papers.

0numbers the graph read from it
0cells of the map it votes in
2citing papers in PubMed
0.2field-weighted citation impact, top 47% 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.

NCT05200715 recruitingnot on this map

Development of an International Multicenter Registry of Patients With Monogenic and Polygenic Autoinflammatory Diseases Aimed at Clinical and Therapeutical Data Collection and Analysis

Typeobservational_patient_registrySponsorUniversity of SienaRan2020 to 2030Enrolled3,500ConditionsHereditary Autoinflammatory Diseases, Schnitzler Syndrome, Behcet Syndrome, PFAPA SyndromeArmsNo intervention is foreseen by the protocol.
3 · Its place in the literature

Who cites it

2 citing papers in PubMed, 4 citations in OpenAlex.

  1. Modulation of adult hippocampal neurogenesis by interleukin 1 signaling.Neurobiology of sleep and circadian rhythms · 2025
    Article
  2. Review
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

28 authors at 13 institutions in 5 countries.

Carla GaggianoResearch Center of Systemic Autoinflammatory Diseases and Behçet's Disease, and Rheumatology-Ophthalmology Collaborative Uveitis Center, Department of Medical Sciences, Surgery and Neurosciences, University of Siena, Siena, Italy; Clinical Pediatrics, Department of Molecular Medicine and Development, University of Siena, Siena, Italy.
Donato RiganteDepartment of Life Sciences and Global Health, Fondazione Policlinico Universitario A. Gemelli IRCCS, Rome, Italy; Rare Diseases and Periodic Fevers Research Centre, Università Cattolica del Sacro Cuore, Rome, Italy.
José Hernández-RodríguezVasculitis Research Unit and Autoinflammatory Diseases Clinical Unit, Department of Autoimmune Diseases, Hospital Clinic of Barcelona, IDIBAPS, University of Barcelona, Barcelona, Spain.
Antonio VitaleResearch Center of Systemic Autoinflammatory Diseases and Behçet's Disease, and Rheumatology-Ophthalmology Collaborative Uveitis Center, Department of Medical Sciences, Surgery and Neurosciences, University of Siena, Siena, Italy.
Maria TarsiaClinical Pediatrics, Department of Molecular Medicine and Development, University of Siena, Siena, Italy.
Alessandra SorianoDepartment of Internal Medicine, Arcispedale Santa Maria Nuova-IRCCS, Reggio Emilia, Italy.
Giuseppe LopalcoRheumatology Unit, Department of Emergency and Organ Transplantation, University of Bari, Bari, Italy.
Florenzo IannoneRheumatology Unit, Department of Emergency and Organ Transplantation, University of Bari, Bari, Italy.ORCID https://orcid.org/0000-0003-0474-5344
Masen Abdel JaberRheumatology Unit, Santa Chiara Hospital, Trento, Italy.
Roberto GiacomelliRheumatology Unit, Department of Biotechnological and Applied Clinical Sciences, University of L'Aquila, L'Aquila, Italy.
Ewa Wiȩsik-SzewczykDepartment of Internal Medicine, Pulmonology, Allergy and Clinical Immunology, Central Clinical Hospital of the Ministry of National Defense, Military Institute of Medicine, Warsaw, Poland.ORCID https://orcid.org/0000-0001-8509-4453
Marco CattaliniPaediatric Clinic, University of Brescia and Spedali Civili di Brescia, Brescia, Italy.
Micol FrassiRheumatology and Clinical Immunology, Spedali Civili, Brescia, Italy; Department of Clinical and Experimental Sciences, University of Brescia, Brescia, Italy.
Matteo PigaRheumatology Unit, Department of Medical Sciences, University and AOU of Cagliari, Cagliari, Italy.
Gaafar RagabRheumatology and Clinical Immunology Unit, Internal Medicine Department, Faculty of Medicine, Cairo University, Cairo, Egypt.
Jurgen SotaResearch Center of Systemic Autoinflammatory Diseases and Behçet's Disease, and Rheumatology-Ophthalmology Collaborative Uveitis Center, Department of Medical Sciences, Surgery and Neurosciences, University of Siena, Siena, Italy.
Fiammetta ZunicaPaediatric Clinic, University of Brescia and Spedali Civili di Brescia, Brescia, Italy.
Alberto FlorisRheumatology Unit, AOU University Clinic, Cagliari, Italy.
Vito SabatoFaculty of Medicine and Health Sciences, Department of Immunology-Allergology-Rheumatology, University of Antwerp and Antwerp University Hospital, Antwerpen, Belgium.
Mohamed Tharwat HegazyRheumatology and Clinical Immunology Unit, Internal Medicine Department, Faculty of Medicine, Cairo University, Cairo, Egypt.ORCID https://orcid.org/0000-0002-2939-5611
Olga AraújoVasculitis Research Unit and Autoinflammatory Diseases Clinical Unit, Department of Autoimmune Diseases, Hospital Clinic of Barcelona, IDIBAPS, University of Barcelona, Barcelona, Spain.
Laura PelegrínClinical Institute of Ophthalmology, Hospital Clinic of Barcelona, IDIBAPS, University of Barcelona, Barcelona, Spain.
Alessandra FabbianiMedical Genetics, University of Siena, Siena, Italy.
Alessandra RenieriMedical Genetics, University of Siena, Siena, Italy; Medical Genetics, Azienda Ospedaliera Universitaria Senese, Siena, Italy.
Salvatore GrossoClinical Pediatrics, Department of Molecular Medicine and Development, University of Siena, Siena, Italy.
Claudia FabianiOphthalmology Unit, Department of Medicine, Surgery and Neurosciences, University of Siena, Siena, Italy.
Bruno FredianiResearch Center of Systemic Autoinflammatory Diseases and Behçet's Disease, and Rheumatology-Ophthalmology Collaborative Uveitis Center, Department of Medical Sciences, Surgery and Neurosciences, University of Siena, Siena, Italy.
Luca CantariniResearch Center of Systemic Autoinflammatory Diseases, Behçet's Disease Clinic and Rheumatology-Ophthalmology Collaborative Uveitis Center, Department of Medical Sciences, Surgery and Neurosciences, University of Siena, Policlinico 'Le Scotte', viale Bracci n. 1, 53100 Siena, Italy.ORCID https://orcid.org/0000-0002-7352-1275
University of Siena · ITAzienda Socio Sanitaria Territoriale degli Spedali Civili di Brescia · ITCairo University · EGConsorci Institut D'Investigacions Biomediques August Pi I Sunyer · ESUniversity of Bari Aldo Moro · ITUniversity of Cagliari · ITCentral Clinical Hospital · PLHospital Clínic de Barcelona · ESOspedale Santa Chiara · ITOspedale Santa Maria · ITUniversità Cattolica del Sacro Cuore · ITUniversity of Antwerp · BEUniversity of L'Aquila · IT

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

backgroundThis study aims at describing the therapeutic outcome of patients carrying the R92Q variant in the

methodsClinical data of patients treated with ANA or CAN for recurrent inflammatory attacks due to the presence of the R92Q variant were retrospectively collected and analysed.

resultsData about 20 treatment courses with IL-1 inhibitors (16 with ANA and 4 with CAN) from 19 patients were collected. Mean age at disease onset was 20.2 ± 14.8 years. In 5 cases (26%) the R92Q variant was found in a family member affected by recurrent fever. The therapeutic response was complete in 13(68%) and partial in 2 patients (11%); treatment failure was observed in 4 cases (21%). Median AIDAI decreased from 10 (interquartile range [IQR] = 28) to 0 (IQR = 1) at the 12-month follow-up visit (

conclusionsR92Q patients showed a favourable response to ANA and CAN, particularly when the mutation segregated in a family member and when a relapsing-remitting disease course or TNF-α receptor-associated periodic syndrome (TRAPS) typical skin rash were observed. In the subgroup of patients not taking advantage of IL-1 blockage different molecular mechanisms underlying the autoinflammatory picture are likely to exist.

Indexed as

anakinrabiologic therapycanakinumabinnovative biotechnologiesinterleukin-1 inhibitionpersonalized medicineR92Q variantTNF-α receptor associated periodic syndrome

Identifiers

PMID34527082
PMCPMC8436281
OpenAlexW3198819780

What OpenQuestion holds

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Registered trials

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.