Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Multicenter Study
. 2025 Jan 8;27(1):5.
doi: 10.1186/s13075-024-03471-x.

Disease activity in patients with idiopathic inflammatory myopathy according to time since diagnosis and positivity to antisynthetase autoantibodies: data from the Myo-Spain registry

Tatiana Cobo-Ibáñez  1   2 Ivan Castellví  3 Ana Pros  4 Marta Domínguez-Álvaro  5 Laura Nuño-Nuño  6 Julia Martínez-Barrio  7 Vega Jovaní  8 Fredeswinda Romero-Bueno  9 Esther Ruiz-Lucea  10 Eva Tomero  11 Ernesto Trallero-Araguás  12 Javier Narváez  13 Jordi Camins-Fàbregas  14 Alberto Ruiz-Román  15 Jesús Loarce-Martos  16 Susana Holgado-Pérez  17 V Miguel Flores-Rodríguez  18 Francisca Sivera  19 Carolina Merino-Argumanez  20 Antonio Juan-Mas  21 Irene Altabás-González  22   23 María Martín-López  24 Joaquín María Belzunegui-Otano  25 Carmen Carrasco-Cubero  26 Mercedes Freire-González  27 Iñigo Rúa-Figueroa  28 Nuria Lozano-Rivas  29 Julio David Suarez-Cuba  30 Olga Martínez  31 Rafaela Ortega-Castro  32 Patricia Alcocer  33 Alejandro Gómez-Gómez  34 Olga Sánchez-Pernaute  9 José Luis Tandaipan  3 Irene Carrión-Barberà  4 Chamaida Plasencia-Rodríguez  6 Oihane Ibarguengoitia-Barrena  10 Paola Vidal-Montal  13 Vera Ortiz-Santamaria  14 Noemi Garrido-Puñal  15 Anne Riveros  17 Esmeralda Delgado-Frías  18 Juan Miguel López-Gómez  19 Carmen Barbadillo  20 José María Pego-Reigosa  22   23 Beatriz E Joven-Ibáñez  24 Jesús Alejandro Valero-Jaimes  25 Elena Naveda  28 Ana Isabel Turrión-Nieves  31 Daniel Seoane-Mato  5 Francisco Javier Prado-Galbarro  5   35 M Ángeles Puche-Larrubia  32
Affiliations
Multicenter Study

Disease activity in patients with idiopathic inflammatory myopathy according to time since diagnosis and positivity to antisynthetase autoantibodies: data from the Myo-Spain registry

Tatiana Cobo-Ibáñez et al. Arthritis Res Ther. .

Abstract

Objective: To evaluate the main outcomes of disease activity and their association with other measures of activity, damage, and quality of life in patients with idiopathic inflammatory myopathy (IIM) according to time since diagnosis and positivity to antisynthetase autoantibodies (ASAs).

Methods: Cross-sectional multicenter study within the Spanish Myo-Spain registry. Cases were classified as incident (≤ 12 months since diagnosis) and prevalent. The main outcomes of disease activity were the Myositis Disease Activity Assessment visual analogue scale (MYOACT), the Manual Muscle Test 8 (MMT-8), physician global activity (PhGA), and extramuscular activity. Other measures of activity, damage, and quality of life included patient global disease activity, MYOACT muscular, creatine phosphokinase, Health Assessment Questionnaire, physician and patient global damage, global damage of the Myositis Damage Index, and the 12-item Short-Form Health Survey (SF-12). We analyzed associations using a multivariate generalized linear model and a simple linear regression model.

Results: A total of 554 patients with different diagnostic subgroups of IIM were included (136 incident and 418 prevalent cases), with 215 ASA-positive patients (58 incident and 157 prevalent cases). All measures of disease activity were higher in the incident cases (p < 0.05), except for MYOACT muscular and creatine phosphokinase, for which no differences were recorded in ASA-positive patients. No differences were found between incident and prevalent cases for measures of damage. Values for the physical component of the SF-12 were higher in the prevalent cases (p < 0.05). The multivariate model was initially significant overall for the main activity outcomes. Positivity to ASAs was positively and negatively associated with the MYOACT index and MMT-8, respectively (p < 0.05), although no association was recorded with PhGA and extramuscular activity. Prevalent cases were negatively associated with the main outcomes of activity, except with MMT-8, for which the association was positive (p < 0.05).

Conclusions: The main activity outcomes validated in polymyositis and dermatomyositis could also be used in other subtypes of IIM, such as antisynthetase syndrome. Recent diagnosis is associated with greater disease activity, as assessed based on these activity outcomes. PhGA and extramuscular activity are not modified by ASA positivity, thus supporting their preferred use for assessing treatment response in IIM with ASAs.

Keywords: Activity; Antisynthetase; Autoantibodies; Damage; Idiopathic inflammatory myopathies.

PubMed Disclaimer

Conflict of interest statement

Declaration. Ethics approval and consent to participate: The study was approved by the reference Clinical Investigation Ethics Committee (Hospital La Paz, Madrid) and by the local ethics committees. All the patients gave their written informed consent before being included in the study. Consent for publication: Not applicable. Competing interests: The authors declare no competing interests.

Figures

Fig. 1
Fig. 1
Manifestations in IIM patients with antisynthetase autoantibodies

References

    1. Sultan SM, Allen E, Oddis CV, Kiely P, Cooper RG, Lundberg IE, et al. Reliability and validity of the myositis disease activity assessment tool. Arthritis Rheum. 2008;58:3593–9. - PubMed
    1. Rider LG, Werth VP, Huber AM, Alexanderson H, Rao AP, Ruperto N, et al. Measures of adult and juvenile dermatomyositis, polymyositis, and inclusion body myositis: Physician and Patient/Parent Global Activity, Manual Muscle Testing (MMT), Health Assessment Questionnaire (HAQ)/Childhood Health Assessment Questionnaire (C-HAQ), Childhood Myositis Assessment Scale (CMAS), Myositis Disease Activity Assessment Tool (MDAAT), Disease Activity Score (DAS), Short Form 36 (SF-36), Child Health Questionnaire (CHQ), physician global damage, Myositis Damage Index (MDI), Quantitative Muscle Testing (QMT), Myositis Functional Index-2 (FI-2), Myositis Activities Profile (MAP), Inclusion Body Myositis Functional Rating Scale (IBMFRS), Cutaneous Dermatomyositis Disease Area and Severity Index (CDASI), Cutaneous Assessment Tool (CAT), Dermatomyositis Skin Severity Index (DSSI), Skindex, and Dermatology Life Quality Index (DLQI). Arthritis Care Res (Hoboken). 2011;63(Suppl 11):118–57. - PMC - PubMed
    1. Benveniste O, Rider LG, ENMC Myositis Outcomes Study Group. 213th ENMC International Workshop: outcome measures and clinical trial readiness in idiopathic inflammatory myopathies, Heemskerk, The Netherlands, 18–20 September 2015. Neuromuscul Disord. 2016;26:523 – 34. - PMC - PubMed
    1. Aggarwal R, Rider LG, Ruperto N, Bayat N, Erman B, Feldman BM, et al. 2016 American College of Rheumatology/European League against Rheumatism criteria for minimal, moderate, and major clinical response in adult dermatomyositis and polymyositis: an International Myositis Assessment and Clinical Studies Group/Paediatric Rheumatology International Trials Organisation Collaborative Initiative. AnnRheum Dis. 2017;76:792–801. - PMC - PubMed
    1. Espinosa-Ortega F, Holmqvist M, Dastmalchi M, Lundberg IE, Alexanderson H. Factors Associated with Treatment response in patients with idiopathic inflammatory myopathies: a Registry-based study. Arthritis Care Res (Hoboken). 2022;74:468–77. - PMC - PubMed

Publication types

LinkOut - more resources