Myopathy

Myopathy associated with systemic sclerosis (SSc)

EUSTAR-SCTC sub-cohort initiative

Britta Maurer1, Rucsandra Dobrota1, Veronika Jaeger2, Lorinda Chung3, Vivien Hsu4, Ulrich A Walker2, Oliver Distler1, on behalf of EUSTAR-SCTC

1Division of Rheumatology, University Hospital Zurich

2Division of Rheumatology, University Hospital Basel

3Division of Rheumatology and Immunology, Stanford University, CA, USA

4Division of Rheumatology, Adult Clinical Research Center, Rutgers-RWJ Medical School, New Brunswick, NJ, USA

Background

In SSc, muscle disease is common (5-96%) and results in significant disability. The varying prevalence (5-96%) reflects the lack of diagnostic consensus criteria and established diagnostic tools.

Objectives

The joint EUSTAR-SCTC initiative addresses those unmet needs by evaluating the clinical course, key features and prognosis of SSc-associated myopathy in a large multicenter international observational SSc cohort.

Methods

Establishment of a SSc-myopathy sub-cohort

  • by extending the EUSTAR MEDS online for myositis-related items
  • based on extensive literature review and expert opinion.

Inclusion criteria (≥ 1)

  • elevated serum levels of muscle enzymes or
  • proximal muscle weakness on physical examination or 
  • muscle atrophy on physical examination or
  • positive myositis-associated autoantibodies.

Collection of biosamples in parallel, yet independently from the observational study.

Interim Analysis October 2015

  • 161 patients from 22 centers enrolled (17 Europe, 4 US, 1 South America)
  • Approx. 100 patients with ≥ 1 follow up visit(s)

Future Activities (next 12 months)

Further expansion of the EUSTAR-SCTC myopathy sub-cohort with

  • longitudinal data collection and linked biosampling in order to define
    • clinical course, key features and prognosis
    • pathophysiologic mechanisms and biomarkers
    • appropriate diagnostic tools
    • treatment strategies.

How to get involved?

Join EUSTAR (http://www.eustar.org/)

  • Templates for ethics approval and patients’ information available
  • Check “participate myopathy study” on the EUSTAR web-page

Contact Britta Maurer (britta.maurer@usz.ch) on behalf of the EUSTAR-SCTC steering committee.