IMPROVEMENT – Improving the outcome in myositis spectrum diseases: core set variables harmonization and use from children to adulthood

Concept

Myositis spectrum disorders (MSDs) inclu- de a wide range of conditions deeply affecting patients’ prognosis and quality of life. Health problems related to MSDs include not only muscle (myositis), but also joints (arthritis/arthralgias), skin (typical cuta- neous lesions) and lungs (Interstitial lung disease).

The timing of onset of different MSDs’findings is generally variable and the risk of a not proper patients’ classification is very high. The myositis expert community recognizes that other steps are necessary for the clarification of different MSD patterns (in both adulthood and childhood), instrumental and laboratory tests to apply and best treatment options.

These steps are mandatory to improve patients’ survival and quality of life, paying special attention to a very vulnerable period for pediatric patients carrying a chronic illness: the transition to an adult age.

Facts and figures

Project lead
Dr L Cavagna
Policlinico S.Matteo Foundation

Dr H Chinoy
University of Manchester
lorenzo.cavagna@unipv.it, hector.chinoy@manchester.ac.uk
FOREUM research grant: € 300.000
2017 - 2020

Meet the team

Dr H Chinoy
University of Manchester
Dr L Cavagna
Policlinico S.Matteo Foundation
Prof L Wedderburn
University College London
Prof M Gonzalez-Gay
Hospital Universitario Marqués de Valdecilla
Mr. U Viora
Associazione Nazionale Malati Reumatici ANMAR

Objectives

To harmonize the international MSDs registries EUMYONET and AENAS with national registries and hospital records; to create a longitudinal database to improve patients’ follow-up, treatment and prognosis.

Performed steps

Systemic literature review for the identification of a first list of eligible variables.

2019 Ongoing steps

  • Eligible variables list submission to collaborating centers and experts for an in depth analysis and a possible up to date (clinicians and patients)
  • Answers collection and analysis
  • Preliminary criteria core set evaluation from steering committee members
  • Final working group for the definition of the final core set of items (March-April 2019 in Pavia)

Other studies supported with the IMPROVEMENT project:

  • ACR/EULAR Classification Criteria of antisynthetase syndrome (CLASS Project):
    we defined the list of participating centers, steering committee members, and the list of variables that should be included in the CRF
  • The anti-MDAS antibodies project:
    characterization of anti-MDA5 antibodies positive patients in a non-Asian cohort. First paper submitted (analysis of 149 patients)
  • The EARTH project (Early myositis Antibodies detection in Recent onset arthritis):
    addressed to evaluate the prevalence of myositis antibodies in a new setting, frequently overlapping with MSDs. Antibodies determination (target of 2.000 patients with early arthritis) is planned for the end of 2019
  • The MyoPAD Study:
    The MyoPAD study aims to integrate mobile health technolo- gies into routine myositis management, to improve recognition of worsening disease activity. The preliminary stages have been planned and data collection has begun.

Patient voice

Patients are involved in every phase of the project. Participants are invited through myositis centres and through already existing registries for myositis. Associazione Nazionale Malati Reumatici (ANMAR), Italy is involved as a patient organisation.

Abstracts EULAR 2019

  • FR0352: Differences in Antisynthetase Syndrome definition and related diagnostic performance. A systematic literature review informing the new ACR/EULAR classification criteria
  • FR0335: Prognostic impact and clinical characteristics of interstitial pneumonia with autoimmune features in a multidisciplinary setting
  • SAT0271: Relationship between Anti-mda5 antibodies and cancer: retrospective analysis of an international and multidisciplinary cohort
  • SAT0286: Evaluation of swallowing in patients with Idiopathic Inflammatory Myopathies