Evidence map›Paper›PMID 37828268›Full record

ArticleInternal and emergency medicine2023

Clinical and laboratory features associated with macrophage activation syndrome in Still's disease: data from the international AIDA Network Still's Disease Registry.

Paola Triggianese, Antonio Vitale, Giuseppe Lopalco, Henrique Ayres Mayrink Giardini, Francesco Ciccia, Ibrahim Al-Maghlouth, Piero Ruscitti, Petros Paul Sfikakis, Florenzo Iannone, Isabele Parente de Brito Antonelli and 69 more

Erratum issuedOpen access · hybridAbstract read
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Article in Internal and emergency medicine, 2023. The graph could read no effect estimate from its abstract, so it casts no vote on the map. An erratum has been issued. Cited by 6 papers.

0numbers the graph read from it
0cells of the map it votes in
6citing papers in PubMed
1.2field-weighted citation impact, top 19% 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

6 citing papers in PubMed, 4 citations in OpenAlex.

  1. Article
  2. Article
  3. Article
  4. Observational
  5. Review
  6. Article
4 · The record

Corrections and comments

5 · Who and what money

Authors and funding

79 authors at 20 institutions in 11 countries.

Paola Triggianese *Rheumatology, Allergology and Clinical Immunology, Department of Systems Medicine, University of Rome Tor Vergata, Rome, Italy.
Antonio Vitale *Department of Medical Sciences, Surgery and Neurosciences, Research Center of Systemic Autoinflammatory Diseases, Behçet's Disease Clinic and Rheumatology-Ophthalmology Collaborative Uveitis Center, University of Siena, Siena, Italy. vitale.reumatologia@gmail.com.
Giuseppe LopalcoRheumatology Unit, Department of Emergency and Organ Transplantation, University of Bari, Bari, Italy.
Henrique Ayres Mayrink GiardiniRheumatology Division, Faculdade de Medicina, Hospital das Clínicas, Universidade de São Paulo, São Paulo, Brazil.
Francesco CicciaDepartment of Precision Medicine, Università Degli Studi Della Campania Luigi Vanvitelli, Naples, Italy.
Ibrahim Al-MaghlouthRheumatology Unit, Department of Medicine, College of Medicine, King Saud University, Riyadh, Saudi Arabia.
Piero RuscittiRheumatology Unit, Department of Biotechnological and Applied Clinical Sciences, University of L'Aquila, L'Aquila, Italy.
Petros Paul SfikakisJoint Academic Rheumatology Program, National and Kapodistrian University of Athens Medical School, Athens, Greece.
Florenzo IannoneRheumatology Unit, Department of Emergency and Organ Transplantation, University of Bari, Bari, Italy.
Isabele Parente de Brito AntonelliRheumatology Division, Faculdade de Medicina, Hospital das Clínicas, Universidade de São Paulo, São Paulo, Brazil.
Martina PatroneDepartment of Precision Medicine, Università Degli Studi Della Campania Luigi Vanvitelli, Naples, Italy.
Kazi Nur AsfinaCollege of Medicine Research Center, College of Medicine, King Saud University, Riyadh, Saudi Arabia.
Ilenia Di ColaRheumatology Unit, Department of Biotechnological and Applied Clinical Sciences, University of L'Aquila, L'Aquila, Italy.
Katerina LaskariJoint Academic Rheumatology Program, National and Kapodistrian University of Athens Medical School, Athens, Greece.
Carla GaggianoDepartment of Medical Sciences, Surgery and Neurosciences, Research Center of Systemic Autoinflammatory Diseases, Behçet's Disease Clinic and Rheumatology-Ophthalmology Collaborative Uveitis Center, University of Siena, Siena, Italy.
Abdurrahman TufanDivision of Rheumatology, Department of Internal Medicine, Gazi University Faculty of Medicine, Ankara, Turkey.
Paolo SfrisoRheumatology Unit, Department of Medicine (DIMED), University of Padova, Padua, Italy.
Lorenzo DagnaUnit of Immunology, Rheumatology, Allergy and Rare Diseases, IRCCS San Raffaele Hospital, Milan, Italy.
Roberto GiacomelliClinical and Research Section of Rheumatology and Clinical Immunology, Fondazione Policlinico Campus Bio-Medico, Via Álvaro del Portillo 200, 00128, Rome, Italy.
Andrea Hinojosa-AzaolaDepartment of Immunology and Rheumatology, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Mexico City, Mexico.
Gaafar RagabInternal Medicine Department, Rheumatology and Clinical Immunology Unit, Faculty of Medicine, Cairo University, Giza, Egypt.
Lampros FotisThird Department of Paediatrics, National and Kapodistrian University of Athens, General University Hospital "Attikon", Athens, Greece.
Haner DireskeneliDepartment of Internal Medicine, Division of Rheumatology, School of Medicine, Marmara University, Istanbul, Turkey.
Veronica SpedicatoRheumatology Unit, Department of Emergency and Organ Transplantation, University of Bari, Bari, Italy.
Marilia Ambiel DagostinRheumatology Division, Faculdade de Medicina, Hospital das Clínicas, Universidade de São Paulo, São Paulo, Brazil.
Daniela IaconoDepartment of Precision Medicine, Università Degli Studi Della Campania Luigi Vanvitelli, Naples, Italy.
Hebatallah Hamed AliCollege of Medicine Research Center, College of Medicine, King Saud University, Riyadh, Saudi Arabia.
Paola CiprianiRheumatology Unit, Department of Biotechnological and Applied Clinical Sciences, University of L'Aquila, L'Aquila, Italy.
Jurgen SotaDepartment of Medical Sciences, Surgery and Neurosciences, Research Center of Systemic Autoinflammatory Diseases, Behçet's Disease Clinic and Rheumatology-Ophthalmology Collaborative Uveitis Center, University of Siena, Siena, Italy.
Riza Can KardasDivision of Rheumatology, Department of Internal Medicine, Gazi University Faculty of Medicine, Ankara, Turkey.
Sara BindoliRheumatology Unit, Department of Medicine (DIMED), University of Padova, Padua, Italy.
Corrado CampochiaroUnit of Immunology, Rheumatology, Allergy and Rare Diseases, IRCCS San Raffaele Hospital, Milan, Italy.
Luca NavariniClinical and Research Section of Rheumatology and Clinical Immunology, Fondazione Policlinico Campus Bio-Medico, Via Álvaro del Portillo 200, 00128, Rome, Italy.
Stefano GentileschiDepartment of Medical Sciences, Surgery and Neurosciences, Research Center of Systemic Autoinflammatory Diseases, Behçet's Disease Clinic and Rheumatology-Ophthalmology Collaborative Uveitis Center, University of Siena, Siena, Italy.
Eduardo Martín-NaresDepartment of Immunology and Rheumatology, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Mexico City, Mexico.
Jiram Torres-RuizDepartment of Immunology and Rheumatology, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Mexico City, Mexico.
Moustafa Ali SaadInternal Medicine Department, Rheumatology and Clinical Immunology Unit, Faculty of Medicine, Cairo University, Giza, Egypt.
Katerina KourtesiThird Department of Paediatrics, National and Kapodistrian University of Athens, General University Hospital "Attikon", Athens, Greece.
Fatma Alibaz-OnerDepartment of Internal Medicine, Division of Rheumatology, School of Medicine, Marmara University, Istanbul, Turkey.
Gizem SevikDepartment of Internal Medicine, Division of Rheumatology, School of Medicine, Marmara University, Istanbul, Turkey.
Annamaria IagnoccoAcademic Rheumatology Center, Dipartimento Scienze Cliniche e Biologiche, Università degli Studi di Torino, Turin, Italy.
Joanna MakowskaDepartment of Rheumatology, Medical University of Lodz, Lodz, Poland.
Marcello GovoniRheumatology Unit, Department of Medical Sciences, Azienda Ospedaliero-Universitaria S. Anna-Ferrara, University of Ferrara, Ferrara, Italy.
Sara MontiRheumatology Department, Istituto di Ricovero e Cura a Carattere Scientifico Policlinico S. Matteo Fondazione, University of Pavia, Pavia, Italy.
Maria Cristina MaggioUniversity Department Pro.Sa.M.I. "G. D'Alessandro", University of Palermo, Palermo, Italy.
Francesco La TorreClinical Pediatrics, University of Bari, Bari, Italy.
Emanuela Del GiudicePediatric and Neonatology Unit, Department of Maternal Infantile and Urological Sciences, Sapienza University of Rome, Polo Pontino, Latina, Italy.
José Hernández-RodríguezVasculitis Research Unit and Autoinflammatory Diseases Clinical Unit, Department of Autoimmune Diseases, Hospital Clinic of Barcelona, IDIBAPS, University of Barcelona, [European Reference Network (ERN) for Rare Immunodeficiency, Autoinflammatory and Autoimmune Diseases (RITA) Center], Barcelona, Spain.
Elena BartoloniDepartment of Medicine and Surgery, MED/16- Rheumatology, Università degli Studi di Perugia, P.Zza Università, 06123, Perugia, Italy.
Giacomo EmmiDepartment of Experimental and Clinical Medicine, University of Florence, Florence, Italy.
Maria Sole ChimentiRheumatology, Allergology and Clinical Immunology, Department of Systems Medicine, University of Rome Tor Vergata, Rome, Italy.
Armin MaierRheumatology Unit, Department of Medicine, Central Hospital of Bolzano, Bolzano, Italy.
Gabriele SimoniniNEUROFARBA Department, Rheumatology Unit, Meyer Children's University Hospital, University of Florence, Florence, Italy.
Giovanni ContiPediatric Nephrology and Rheumatology Unit, AOU Policlinic G Martino, Messina, Italy.
Alma Nunzia OlivieriDepartment of Woman, Child and of General and Specialized Surgery, University of Campania "Luigi Vanvitelli", Naples, Italy.
Maria TarsiaDepartment of Medical Sciences, Surgery and Neurosciences, Research Center of Systemic Autoinflammatory Diseases, Behçet's Disease Clinic and Rheumatology-Ophthalmology Collaborative Uveitis Center, University of Siena, Siena, Italy.
Amato De PaulisSection of Clinical Immunology, Department of Translational Medical Sciences, University of Naples Federico II, Naples, Italy.
Alberto Lo GulloUnit of Rheumatology, Department of Medicine, ARNAS Garibaldi Hospital, Catania, Italy.
Ewa Więsik-SzewczykDepartment of Internal Medicine, Pulmonology, Allergy and Clinical Immunology, Central Clinical Hospital of the Ministry of National Defence, Military Institute of Medicine, National Research Institute, Warsaw, Poland.
Ombretta ViapianaRheumatology Unit, Department of Medicine, University and Azienda Ospedaliera Universitaria Integrata of Verona, Verona, Italy.
Benson OgunjimiAntwerp Unit for Data Analysis and Computation in Immunology and Sequencing, University of Antwerp, Antwerp, Belgium.
Samar TharwatRheumatology and Immunology Unit, Internal Medicine Department, Mansoura University, Mansoura, Egypt.
Sukran ErtenDepartment of Rheumatology, Faculty of Medicine Ankara City Hospital, Ankara Yıldırım Beyazıt University, Ankara, Turkey.
Rossana NuzzoleseDepartment of Medical Sciences, Surgery and Neurosciences, Research Center of Systemic Autoinflammatory Diseases, Behçet's Disease Clinic and Rheumatology-Ophthalmology Collaborative Uveitis Center, University of Siena, Siena, Italy.
Anastasios KaramanakosJoint Academic Rheumatology Program, First Department of Propaedeutic and Internal Medicine, National and Kapodistrian University of Athens, Mikras Asias Street 75 Goudi, 11527, Athens, Greece.
Micol FrassiRheumatology and Clinical Immunology, Spedali Civili and Department of Clinical and Experimental Sciences, University of Brescia, [European Reference Network (ERN) for Rare Immunodeficiency, Autoinflammatory and Autoimmune Diseases (RITA) Center], Brescia, Italy.
Alessandro ConfortiU.O. Medicina Generale, Ospedale San Paolo di Civitavecchia, ASL Roma 4, Civitavecchia, Rome, Italy.
Valeria CaggianoDepartment of Medical Sciences, Surgery and Neurosciences, Research Center of Systemic Autoinflammatory Diseases, Behçet's Disease Clinic and Rheumatology-Ophthalmology Collaborative Uveitis Center, University of Siena, Siena, Italy.
Achille MarinoUnit of Pediatric Rheumatology, ASST Gaetano Pini-CTO, Milan, Italy.
Gian Domenico SebastianiUOC di Reumatologia, Azienda Ospedaliera San Camillo Forlanini, Rome, Italy.
Antonio GidaroDepartment of Biomedical and Clinical Sciences Luigi Sacco, Luigi Sacco Hospital, University of Milan, Milan, Italy.
Enrico TombettiInternal Medicine, Fatebenefratelli Hospital, Milan, Italy.
Francesco CarubbiDepartment of Life, Health & Environmental Sciences and Internal Medicine and Nephrology Unit, Department of Medicine, University of L'Aquila and ASL Avezzano-Sulmona-L'Aquila, San Salvatore Hospital, L'Aquila, Italy.
Giovanni RubegniOphthalmology Unit, Department of Medicine, Surgery and Neurosciences, University of Siena and Azienda Ospedaliero-Universitaria Senese [European Reference Network (ERN) for Rare Immunodeficiency, Autoinflammatory and Autoimmune Diseases (RITA) Center], Siena, Italy.
Alessandra CartocciBioengineering and Biomedical Data Science Lab, Department of Medical Biotechnologies, University of Siena, Siena, Italy.
Alberto BalistreriBioengineering and Biomedical Data Science Lab, Department of Medical Biotechnologies, University of Siena, Siena, Italy.
Claudia FabianiOphthalmology Unit, Department of Medicine, Surgery and Neurosciences, University of Siena and Azienda Ospedaliero-Universitaria Senese [European Reference Network (ERN) for Rare Immunodeficiency, Autoinflammatory and Autoimmune Diseases (RITA) Center], Siena, Italy.
Bruno FredianiDepartment of Medical Sciences, Surgery and Neurosciences, Research Center of Systemic Autoinflammatory Diseases, Behçet's Disease Clinic and Rheumatology-Ophthalmology Collaborative Uveitis Center, University of Siena, Siena, Italy.
Luca CantariniDepartment of Medical Sciences, Surgery and Neurosciences, Research Center of Systemic Autoinflammatory Diseases, Behçet's Disease Clinic and Rheumatology-Ophthalmology Collaborative Uveitis Center, University of Siena, Siena, Italy.ORCID http://orcid.org/0000-0002-7352-1275
University of Siena · ITNational and Kapodistrian University of Athens · GRUniversity of Bari Aldo Moro · ITUniversity of Campania "Luigi Vanvitelli" · ITUniversity of L'Aquila · ITHospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo · BRInstituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán · MXKing Saud University · SAMarmara University · TRCairo University · EGGazi University · TRUniversità Campus Bio-Medico · ITUniversity of Milan · ITUniversity of Padua · ITUniversity of Rome Tor Vergata · ITVita-Salute San Raffaele University · ITAnkara Yıldırım Beyazıt University · TRASL Roma · ITAzienda Ospedaliera San Camillo-Forlanini · ITAzienda Ospedaliera Universitaria Integrata Verona · IT

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

To characterize clinical and laboratory signs of patients with Still's disease experiencing macrophage activation syndrome (MAS) and identify factors associated with MAS development. Patients with Still's disease classified according to internationally accepted criteria were enrolled in the AutoInflammatory Disease Alliance (AIDA) Still's Disease Registry. Clinical and laboratory features observed during the inflammatory attack complicated by MAS were included in univariate and multivariate logistic regression analysis to identify factors associated to MAS development. A total of 414 patients with Still's disease were included; 39 (9.4%) of them developed MAS during clinical history. At univariate analyses, the following variables were significantly associated with MAS: classification of arthritis based on the number of joints involved (p = 0.003), liver involvement (p = 0.04), hepatomegaly (p = 0.02), hepatic failure (p = 0.01), axillary lymphadenopathy (p = 0.04), pneumonia (p = 0.03), acute respiratory distress syndrome (p < 0.001), platelet abnormalities (p < 0.001), high serum ferritin levels (p = 0.009), abnormal liver function tests (p = 0.009), hypoalbuminemia (p = 0.002), increased LDH (p = 0.001), and LDH serum levels (p < 0.001). At multivariate analysis, hepatomegaly (OR 8.7, 95% CI 1.9-52.6, p = 0.007) and monoarthritis (OR 15.8, 95% CI 2.9-97.1, p = 0.001), were directly associated with MAS, while the decade of life at Still's disease onset (OR 0.6, 95% CI 0.4-0.9, p = 0.045), a normal platelet count (OR 0.1, 95% CI 0.01-0.8, p = 0.034) or thrombocytosis (OR 0.01, 95% CI 0.0-0.2, p = 0.008) resulted to be protective. Clinical and laboratory factors associated with MAS development have been identified in a large cohort of patients based on real-life data.

Indexed as

Liver DiseasesMacrophage Activation SyndromeStill's Disease, Adult-OnsetHepatomegalyHumansArthritisAutoinflammatory diseasesDiagnosisMASPrognosis

Identifiers

PMID37828268
PMCPMC10635948
OpenAlexW4387580580

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