A Christensen1, S Khalique2, S Cenac3, K Fligelstone4, A Mawdsley5, T French6, J K Gordon7, M Baron8, E Busman9, V D Steen10, L A Saketkoo11. 1. Tulane University School of Medicine, New Orleans, LA. 2. Louisiana University Health Sciences Center, New Orleans, LA. 3. Louisiana University Health Sciences Center, New Orleans. 4. Royal Free Hospital, Scleroderma Unit and The Scleroderma Society UK, London, UK. 5. Raynaud's and Scleroderma - Care and Support, UK. 6. University of Utah, Salt Lake City, UT. 7. Hospital for Special Surgery, NYC, NY. 8. Jewish General Hospital, McGill University, Montreal, Canada. 9. Atlanta Scleroderma Support Group, Atlanta, GA. 10. Georgetown University Medical Center, Washington, DC. 11. University Medical Center Scleroderma and Sarcoidosis Patient Care and Research Center, New Orleans.
Abstract
BACKGROUND: Calcinosis is a disabling, rarely discussed manifestation of systemic sclerosis (SSc) for which the natural history and management is understood poorly. OBJECTIVES: To develop a calcinosis specific patient reported outcome measure (PROM) that can be used for future clinical research to test the effects of therapy on scleroderma related calcinosis. METHODS: Patients were selected for participation by their scleroderma physicians. Four focus groups and individual interviews were recorded and transcribed verbatim. Patients were asked to frame questions to help a physician learn if calcinosis was better, worse or the same. Patient transcripts underwent an iterative inductive process (no preconceived coding, content drives coding and analysis) by at least five 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) consented and were interviewed. Responses included concepts of self-management strategies and recurrent hypotheses relating calcinosis development to trauma, Raynaud's and cold exposure. We identified discrete concepts such as the perceived association between cold exposure, Raynaud's and calcinosis severity. Calcinosis tended to present along with or soon after SSc diagnosis and remained throughout disease duration - though was not yet compared to report of first Raynaud experience. CONCLUSIONS: Patient observations and self-management behavior provide opportunities for experts to learn from and to preemptively educate physicians and patients. Patients are eager for self-management guidance. These concepts are the groundwork for PROM development. However, patients suggested a composite of scales anchored in pain, size, frequency, number and related impairment may reasonably serve as an interim instrument for SSc calcinosis.
BACKGROUND:Calcinosis is a disabling, rarely discussed manifestation of systemic sclerosis (SSc) for which the natural history and management is understood poorly. OBJECTIVES: To develop a calcinosis specific patient reported outcome measure (PROM) that can be used for future clinical research to test the effects of therapy on scleroderma related calcinosis. METHODS:Patients were selected for participation by their scleroderma physicians. Four focus groups and individual interviews were recorded and transcribed verbatim. Patients were asked to frame questions to help a physician learn if calcinosis was better, worse or the same. Patient transcripts underwent an iterative inductive process (no preconceived coding, content drives coding and analysis) by at least five 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) consented and were interviewed. Responses included concepts of self-management strategies and recurrent hypotheses relating calcinosis development to trauma, Raynaud's and cold exposure. We identified discrete concepts such as the perceived association between cold exposure, Raynaud's and calcinosis severity. Calcinosis tended to present along with or soon after SSc diagnosis and remained throughout disease duration - though was not yet compared to report of first Raynaud experience. CONCLUSIONS:Patient observations and self-management behavior provide opportunities for experts to learn from and to preemptively educate physicians and patients. Patients are eager for self-management guidance. These concepts are the groundwork for PROM development. However, patients suggested a composite of scales anchored in pain, size, frequency, number and related impairment may reasonably serve as an interim instrument for SSc calcinosis.
Authors: Lesley Ann Saketkoo; Tracy Frech; Cecília Varjú; Robyn Domsic; Jessica Farrell; Jessica K Gordon; Carina Mihai; Nora Sandorfi; Lee Shapiro; Janet Poole; Elizabeth R Volkmann; Monika Lammi; Kendra McAnally; Helene Alexanderson; Henrik Pettersson; Faye Hant; Masataka Kuwana; Ami A Shah; Vanessa Smith; Vivien Hsu; Otylia Kowal-Bielecka; Shervin Assassi; Maurizio Cutolo; Cristiane Kayser; Victoria K Shanmugam; Madelon C Vonk; Kim Fligelstone; Nancy Baldwin; Kerri Connolly; Anneliese Ronnow; Beata Toth; Maureen Suave; Sue Farrington; Elana J Bernstein; Leslie J Crofford; László Czirják; Kelly Jensen; Monique Hinchclif; Marie Hudson; Matthew R Lammi; Jennifer Mansour; Nadia D Morgan; Fabian Mendoza; Mandana Nikpour; John Pauling; Gabriela Riemekasten; Anne-Marie Russell; Mary Beth Scholand; Elise Seigart; Tatiana Sofia Rodriguez-Reyna; Laura Hummers; Ulrich Walker; Virginia Steen Journal: Best Pract Res Clin Rheumatol Date: 2021-09-15 Impact factor: 4.991
Authors: Murray Baron; Bashar Kahaleh; Elana J Bernstein; Lorinda Chung; Philip J Clements; Christopher Denton; Robyn T Domsic; Nava Ferdowsi; Ivan Foeldvari; Tracy Frech; Jessica K Gordon; Marie Hudson; Sindhu R Johnson; Dinesh Khanna; Zsuzsannah McMahan; Peter A Merkel; Sonali Narain; Mandana Nikpour; John D Pauling; Laura Ross; Antonia Maria Valenzuela Vergara; Alessandra Vacca Journal: J Scleroderma Relat Disord Date: 2018-07-18
Authors: Tracy M Frech; John M VanBuren; Emily Startup; Shervin Assassi; Elana J Bernstein; Flavia V Castelino; Lorinda Chung; Chase Correia; Jessica K Gordon; Faye N Hant; Laura Hummers; Dinesh Khanna; Nora Sandorfi; Ami A Shah; Victoria K Shanmugam; Virginia Steen; Luke Evnin Journal: Clin Rheumatol Date: 2020-10-23 Impact factor: 2.980