Patient Experience of Systemic Sclerosis-Related Calcinosis: An International Study Informing Clinical Trials, Practice, and the Development of the Mawdsley Calcinosis Questionnaire

Lesley Ann Saketkoo; Jessica K Gordon; Kim Fligelstone; Anne Mawdsley; Humza A Chaudhry; Antonia Valenzuela; Angela Christensen; Samara M Khalique; Kelly Jensen; Sophia C Weinmann; Evan Busman; Lorinda Chung; Vivien M Hsu; Anne-Marie Russell; Virginia D Steen

doi:10.1016/j.rdc.2023.01.017

Patient Experience of Systemic Sclerosis-Related Calcinosis: An International Study Informing Clinical Trials, Practice, and the Development of the Mawdsley Calcinosis Questionnaire

Rheum Dis Clin North Am. 2023 May;49(2):463-481. doi: 10.1016/j.rdc.2023.01.017.

Authors

Affiliations

¹ New Orleans Scleroderma and Sarcoidosis Patient Care and Research Center, New Orleans, LA, USA; University Medical Center-Comprehensive Pulmonary Hypertension Center and Interstitial Lung Disease Clinic Programs, New Orleans, LA, USA; Section of Pulmonary Medicine, Louisiana State University School of Medicine, New Orleans, LA, USA; Tulane University School of Medicine, New Orleans, LA, USA. Electronic address: lsaketk@tulane.edu.
² Department of Rheumatology, Hospital for Special Surgery, New York, NY, USA.
³ Scleroderma & Raynaud Society, UK (SRUK); Federation of European Scleroderma Associations, UK.
⁴ Raynaud's & Scleroderma Association-Care and Support, London, UK.
⁵ New Orleans Scleroderma and Sarcoidosis Patient Care and Research Center, New Orleans, LA, USA; Tulane University School of Medicine, New Orleans, LA, USA.
⁶ Department of Rheumatology and Clinical Immunology, Pontificia Universidad Católica de Chile, Santiago, Chile.
⁷ Doctors Hospital, Renaissance, TX, USA.
⁸ Department of Rheumatology, Virginia Tech Carilion Clinic School of Medicine, Roanoke, VA, USA.
⁹ New Orleans Scleroderma and Sarcoidosis Patient Care and Research Center, New Orleans, LA, USA; Tulane University School of Medicine, New Orleans, LA, USA; University of Colorado School of Medicine, Denver, CO, USA.
¹⁰ Department of Medicine, Division of Rheumatology and Immunology, Duke University Hospital, Durham, NC, USA.
¹¹ Healthcare Patient Advocate, Atlanta, GA, USA.
¹² Department of Medicine, Division of Immunology and Rheumatology, Stanford University School of Medicine and Palo Alto VA Healthcare System, Palo Alto, CA, USA; Department of Dermatology, Division of Immunology and Rheumatology, Stanford University School of Medicine and Palo Alto VA Healthcare System, Palo Alto, CA, USA.
¹³ RWJ-Scleroderma Program, Rutgers Robert Wood Johnson Medical School, New Brunswick, NJ, USA.
¹⁴ Respiratory Institute, University of Exeter, Exeter, UK; Respiratory Medicine, Royal Devon University Healthcare NHS Foundation Trust, UK.
¹⁵ Division of Rheumatology, Department of Medicine, Georgetown University, Washington, DC, USA.

PMID: 37028847
DOI: 10.1016/j.rdc.2023.01.017

Abstract

Systemic sclerosis (SSc) -related calcinosis can be a debilitating, constantly painful, poorly understood vascular complication of calcium hydroxyapatite deposition in soft tissue structures that affects approximately 40% of both limited and diffuse cutaneous SSc subtypes. This publication describes the iterative and multitiered international qualitative investigations that yielded remarkable insights into natural history, daily experience, and complications of SSc-calcinosis providing pivotal information for health management. Patient-driven question development and field testing, according to Food and Drug Administration guidance, propelled the development of a patient-reported outcome measure for SSc-calcinosis, the Mawdsley Calcinosis Questionnaire.

Keywords: Calcinosis; Digital ulcers; Patient-reported outcomes; Qualitative research; Qualtiy of LIfe; Raynaud phenomenon; Scleroderma; Self-Management.

Publication types

Review

MeSH terms

Calcinosis* / complications
Humans
Patient Outcome Assessment
Scleroderma, Systemic* / complications
Surveys and Questionnaires