Myopathy Working Group

Summary of goals/project(s) of working group:

1. Exploring systemic sclerosis-associated myopathy: Systematic Literature Review

This project will synthesize existing evidence on the definition, diagnosis, screening, treatment, and monitoring of SSc-associated myopathy. It will follow rigorous methodology, including predefined inclusion/exclusion criteria (e.g., English-language articles only, excluding abstracts and single case reports), dual-reviewer screening, and structured data extraction. Two SLRs are anticipated: 1) one focusing on definition, diagnosis and screening, and 2) another addressing treatment and monitoring. Findings will identify gaps in the literature and inform the subsequent Delphi consensus process.

2. Consensus-based approach to systemic sclerosis-associated myopathy: Delphi Consensus Exercise

Building on the literature reviews, a Delphi exercise will be conducted to achieve international expert consensus on key elements of a proposed definition, diagnosis, screening, treatment, and monitoring. Multiple rounds of surveys will refine definition/clinical features, diagnostic/screening thresholds, and treatment/monitoring strategies.

3. Additional Research Initiatives

Future projects will enhance understanding of the clinical and pathological features of myopathy in SSc—including its presentation, progression, and differentiation from other disease manifestations. These may include the development of a registry or database of SSc patients with myopathy, as well as collaborative translational studies—such as autoantibody profiling and biomarker discovery—to inform the design of future clinical trials.

Progress to date for working group:

1. Systematic literature review: We assembled the SLR team, who is currently building search strategies.
2. Delphi consensus exercise: Currently, in the preparation phase. We have built main research questions and selected Delphi exercise coordinators.

Next steps:

1. Systematic literature review:

• Reference selection, data extraction, quality assessment.
• Summarize findings and submit for publication.

2. Delphi consensus exercise:

• Design first questionnaire.
• Move forward to Consultation phase

What are expected deliverables and timesline for grant/working group?

• October 2025: In-person meeting at the ACR/SCTC conference to engage broader interest and prioritize project focus.
• February 2026: Present SLR findings and build a summary to inform Delphi exercise.
• April 2026: Submit funding application for the selected project based on feedback from the ACR/SCTC and SCTC collective WG meeting.

Contact information:

• Antonia Valenzuela: valenzuela@uc.cl
• Lorinda S Chung: shauwei@stanford.edu  
• Oliver Distler: Distler@usz.ch
• Julie Paik: jpaik1@jhmi.edu
• Julian Esteban Barahona Correa (WG Fellow): jbarahon@stanford.edu