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Clinical spectrum time course in non-Asian patients positive for anti-MDA5 antibodies

2022· article· en· W4214779842 on OpenAlexaff
Lorenzo Cavagna, Federica Meloni, Alain Meyer, Gianluca Sambataro, Mirko Belliato, Ellen De Langhe, Ilaria Cavazzana, Nicolò Pipitone, Konstantinos Triantafyllias, Marta Mosca, Simone Barsotti, G. Zampogna, Alessandro Biglia, Giacomo Emmi, Anneke J. van der Kooi, Paola Parronchi, Sandrine Hirschi, José António Pereira da Silva, Carlo Alberto Scirè, Federica Furini, Margherita Giannini, Olga Martínez González, Laura Damian, Yves Piette, Vanessa Smith, Antonio Mera-Valera, Javier Bachiller‐Corral, Ivan Cabezas Rodriguez, Anahy Brandy-García, F. Maurier, Julie Perrin, Juan González‐Moreno, Ulrich Drott, Christiane Delbrück, Andreas Schwarting, Eugenio Arrigoni, Gian Domenico Sebastiani, Annamaria Iuliano, Carlotta Nannini, Luca Quartuccio, Ana Belén Rodríguez Cambrón, María Ángeles Blázquez-Cañamero, Ignacio Villa Blanco, Giovanni Cagnotto, Alberto Pesci, Francesco Luppi, Giulia Dei, Fredeswinda I. Romero Bueno, Franco Franceschini, Ilaria Chiapparoli, Giovanni Zanframundo, Sara Lettieri, Ludovico De Stefano, Maurizio Cutolo, Alessandro Mathieu, Matteo Piga, Sergio Prieto‐González, Maria Francisca Moraes‐Fontes, João Eurico Fonseca, Vega Jovaní, Valeria Riccieri, Alessandro Santaniello, Stephen Montfort, David Bilocca, Gian Luca Erre, Elena Bartoloni, Roberto Gerli, Maria Cristina Monti, Hanns M. Lorenz, Domenico Sambataro, Silvia Bellando-Randone, Udo Schneider, Claudia Valenzuela, Raquel López‐Mejías, José M. Cifrián, Mayra Mejía, Monserrat-Ixchel Gonzalez Perez, Sarah K. Wendel, Marco Fornaro, Giacomo De Luca, Giovanni Orsolini, Maurizio Rossini, Philippe Dieudé, Johannes Knitza, Santos Castañeda, Reinhard Voll, Jorge Rojas‐Serrano, Adele Valentini, Carlo Vancheri, Marco Matucci‐Cerinic, Eugen Feist, Veronica Codullo, Florenzo Iannone, Jörg H. W. Distler, Carlomaurizio Montecucco, Miguel Á. González‐Gay

Bibliographic record

VenueClinical and Experimental Rheumatology · 2022
Typearticle
Languageen
FieldMedicine
TopicInflammatory Myopathies and Dermatomyositis
Canadian institutionsUniversity Hospital Foundation
FundersFoundation for Research in Rheumatology
KeywordsMedicineDermatomyositisInterstitial lung diseaseInternal medicineRheumatoid arthritisPneumoniaConnective tissue diseaseGastroenterologyMyositisArthritisLungAutoimmune diseaseDisease

Abstract

fetched live from OpenAlex

OBJECTIVES: To define the clinical spectrum time-course and prognosis of non-Asian patients positive for anti-MDA5 antibodies. METHODS: We conducted a multicentre, international, retrospective cohort study. RESULTS: 149 anti-MDA5 positive patients (median onset age 53 years, median disease duration 18 months), mainly females (100, 67%), were included. Dermatomyositis (64, 43%) and amyopathic dermatomyositis (47, 31%), were the main diagnosis; 15 patients (10%) were classified as interstitial pneumonia with autoimmune features (IPAF) and 7 (5%) as rheumatoid arthritis. The main clinical findings observed were myositis (84, 56%), interstitial lung disease (ILD) (108, 78%), skin lesions (111, 74%), and arthritis (76, 51%). The onset of these manifestations was not concomitant in 74 cases (50%). Of note, 32 (21.5%) patients were admitted to the intensive care unit for rapidly progressive-ILD, which occurred in median 2 months from lung involvement detection, in the majority of cases (28, 19%) despite previous immunosuppressive treatment. One-third of patients (47, 32% each) was ANA and anti-ENA antibodies negative and a similar percentage was anti-Ro52 kDa antibodies positive. Non-specific interstitial pneumonia (65, 60%), organising pneumonia (23, 21%), and usual interstitial pneumonia-like pattern (14, 13%) were the main ILD patterns observed. Twenty-six patients died (17%), 19 (13%) had a rapidly progressive-ILD. CONCLUSIONS: The clinical spectrum of the anti-MDA5 antibodies-related disease is heterogeneous. Rapidly-progressive ILD deeply impacts the prognosis also in non-Asian patients, occurring early during the disease course. Anti-MDA5 antibody positivity should be considered even when baseline autoimmune screening is negative, anti-Ro52 kDa antibodies are positive, and radiology findings show a NSIP pattern.

Fetched live from OpenAlex and de-inverted. Abstracts are not stored in this database: the inverted indexes are 8.6 GB of the frame’s 9.3 GB of text, and the host has 13 GB free.

How this classification was reachedexpand

Full frame distilled prediction

Teacher imitation

Not calibrated prevalence, not ground truth. Human validation pending. Learned from the 10,348 direct Codex labels and 10,348 direct Gemma labels. Candidate is the union of thresholded teacher heads; consensus is their intersection. These outputs are machine_predicted_unvalidated and are not human labels or direct frontier model labels.

metaresearch head score (Codex)0.001
metaresearch head score (Gemma)0.000
Version: codex-gemma-dda1882f352aValidation status: machine_predicted_unvalidated
Candidate categoriesnone
Consensus categoriesnone
DomainCandidate signal: none · Consensus signal: none
Study designCandidate signal: Observational · Consensus signal: Observational
GenreCandidate signal: Empirical · Consensus signal: Empirical
Teacher disagreement score0.017
Threshold uncertainty score0.816

Codex and Gemma teacher scores by category

CategoryCodexGemma
Metaresearch0.0010.000
Meta-epidemiology (narrow)0.0000.000
Meta-epidemiology (broad)0.0010.000
Bibliometrics0.0000.000
Science and technology studies0.0000.001
Scholarly communication0.0000.000
Open science0.0000.000
Research integrity0.0000.000
Insufficient payload (model declined to judge)0.0000.000

Machine scores (provisional)

The two teacher heads of the student model, read on this work. A score orders the frame for review; it never asserts a category, and the validation status ships verbatim with every row.

Baseline scores from an immature model (maturity gate not passed, 7 training rounds). Scores rank; they never assert a category.

Opus teacher head0.013
GPT teacher head0.347
Teacher spread0.334 · how far apart the two teachers sit on this one work
Validation statusscore_only:v0-immature-baseline · verbatim from the scoring run: score_only means the number may rank works, and no category label ships from it

Classification

machine, unvalidated

Machine predicted; a candidate call from one teacher head, not a consensus.

The models applied no category: nothing in the taxonomy fit this work.
Study designObservational
Domainnot available
GenreEmpirical

How this classification was reached, model by model and score by score, is at the end of the page under "How this classification was reached".

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Citations40
Published2022
Admission routes1
Has abstractyes

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