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FRI0475 Systemic Sclerosis (SSC) Related Calcinosis: Patients Provide what Specialists Want to Learn – Development of a Calcinosis Patient Reported Outcome Measure (PROM)
  1. A.M. Christensen1,
  2. S. Khalique2,
  3. S. Cenac2,
  4. K. Fligelstone3,4,
  5. V. Jaeger5,
  6. A. Mawdsley6,
  7. R. Kaufman2,
  8. T. Frech7,
  9. J.K. Gordon8,
  10. V. Steen9,
  11. A. Aubin2,
  12. M. Baron10,
  13. E. Busman11,
  14. L.A. Saketkoo1
  15. on behalf of Scleroderma Clinical Trials Consortium (SCTC) Calcinosis Working Group
  1. 1UMC Scleroderma and Sarcoidosis Patient Care and Research Center, Tulane University
  2. 2LSU Health Sciences Center, New Orleans, United States
  3. 3Scleroderma Society UK
  4. 4Scleroderma Unit, Royal Free Hospital, London, United Kingdom
  5. 5Department of Rheumatology, University of Basel, Basel, Switzerland
  6. 6Rayanuds & Scleroderma Care & Support UK, Cheshire, United Kingdom
  7. 7University of Utah, Salt Lake City, UT
  8. 8Hospital for Special Surgery, New York
  9. 9Georgetown University, Washington DC, United States
  10. 10McGill University, Montreal, Canada
  11. 11Scleroderma Foundation - Support Group Leader, Atlanta, United States

Abstract

Background Calcinosis is a disabling, rarely discussed manifestation of SSc for which the natural history and management is poorly understood. This investigation is the 1st phase of a multi-tiered project to understand calcinosis from patients' perspectives creating the groundwork for a SSc-calcinosis PROM.

Methods Five focus groups and individual interviews in the US and UK were recorded and transcribed verbatim. Pathophysiologic and life impact were elicited with: 1. Since developing calcinosis how has your life changed over time? 2. How has calcinosis changed over time? A final probe was a request for questions that a clinician could ask to understand if calcinosis was better, worse or same.

Transcripts were analysed by hand (highest method) by an iterative inductive process (content drives coding) by at least 5 independent analysts including at least one patient research partner. Concepts were triangulated until a comprehensive set of concepts emerged. Occurrence was quantified per participant.

Results Twenty-three patients (29/31 female, 27/31 white, with mean disease duration 18.1 years) were consented and interviewed. Responses spanned broadly to include concepts of self-management strategies and recurrent hypotheses relating calcinosis development to trauma, Raynaud's and cold exposure (tables 1 & 2).

Cold exposure and Raynaud's were a perceived association to calcinosis severity - “when they are cold mine always open back up”.

A majority of patients engage in strategies to extrude calcinosis with either pressure +/- soaking or at home surgical techniques. “I actually have homemade surgical tools to get these out.”

The following anchors were consistently suggested for physicians to assess calcinosis severity: pain level, size, frequency, number and functional impairment. A 2-step question was suggested to help differentiate ulcer, infection and calcinosis symptoms: 1st regarding predomiant wound character and then target the related calcinosis.

Conclusions These results provide the groundwork for and conclude the 1st steps (item collection) in PROM development. As suggested by patients, a composite of scales anchored in pain, size, frequency, number and related impairment may reasonably serve as an interim instrument for SSc calcinosis. Next steps are validating content with a large subject base and questionniare development with subsequent validation.

Very importantly, patients' observations and self-management behavior provide opportunities to learn from and to preemptively educate physicians and patients. Patients are eager for self-management guidance. An essential deliverable of this work will be a patient-physician education guide on calcinosis management.

Acknowledgements In memory of Anne Mawdsley, founder: Raynaud's & Scleroderma Association UK – tireless engine for education and advocacy raising >£10 million for SSc research.

Disclosure of Interest A. Christensen: None declared, S. Khalique: None declared, S. Cenac: None declared, K. Fligelstone: None declared, V. Jaeger: None declared, A. Mawdsley: None declared, R. Kaufman: None declared, T. Frech: None declared, J. Gordon: None declared, V. Steen: None declared, A. Aubin: None declared, M. Baron: None declared, E. Busman: None declared, L. A. Saketkoo Grant/research support from: ACR/EULAR Exchange Awardee

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