Background/Purpose: Calcinosis is a disabling, rarely discussed manifestation of SSc for which the natural history and management is poorly understood.  Last year, the Scleroderma Clinical Trials Consortium (SCTC) established a task force to develop a calcinosis specific patient reported measure (PROM).  This investigation is the 1^st phase of a multi-tiered project.

Methods: Four focus groups and individual interviews in the US and UK were recorded and transcribed verbatim.  To capture both pathophysiologic and life impact, 2 questions were asked: 1. Since developing calcinosis how has your life changed over time?  2. How has the calcinosis changed over time?  Patients were also asked to frame questions to help a physician learn if calcinosis was better, worse or the same.

Transcripts each underwent an iterative inductive process (no preconceived coding, content drives coding and analysis) by at least 5 independent analysts including at least one research team member with SSc.  Concepts were triangulated to identify a comprehensive set of meaningful concepts with occurrence quantified per participant.

Results:   Twenty-three patients (22/23 female, 19/23 white, with mean disease duration 14.8 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.  We identified discrete concepts which are described in Table 1 along with the proportion of patients declaring personal relevance.

Cold exposure and Raynaud’s were a perceived association to calcinosis severity – “when they are cold mine always open back up”.   Several described a disabling core body phenomenon involving decreased core temperature with a rapid physical decline requiring prolonged recovery potentially lasting hours – “it’s like intense – it racks your whole body”.  Calcinosis tended to present along with or soon after SSc diagnosis and remained throughout disease duration.

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 indicated to assess calcinosis severity:  pain level, size, frequency, number and functional impairment.

Conclusion:    Patient observations and self-management behavior provide opportunities to learn from and to preemptively educate physicians and patients.  Patients are eager for self-management guidance.  These concepts provide the groundwork for PROM development.  However, 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 until that time.