S R Johnson1, M L Soowamber2, J Fransen3, D Khanna4, F Van Den Hoogen3, M Baron5, M Matucci-Cerinic6, C P Denton7, T A Medsger8, P E Carreira9, G Riemekasten10, J Distler11, A Gabrielli12, V Steen13, L Chung14, R Silver15, J Varga16, U Müller-Ladner17, M C Vonk18, U A Walker19, F A Wollheim20, A Herrick21, D E Furst22, L Czirjak23, O Kowal-Bielecka24, F Del Galdo25, M Cutolo26, N Hunzelmann27, C D Murray28, I Foeldvari29, L Mouthon30, N Damjanov31, B Kahaleh32, T Frech33, S Assassi34, L A Saketkoo35, J E Pope36. 1. a Toronto Scleroderma Program, Division of Rheumatology, Department of Medicine, Toronto Western Hospital, Institute of Health Policy, Management and Evaluation , University of Toronto , Toronto , ON , Canada. 2. b Toronto Scleroderma Program, Division of Rheumatology, Department of Medicine, Toronto Western Hospital , University of Toronto , Toronto , ON , Canada. 3. c The Radboud University Nijmegen Medical Centre , Nijmegen , The Netherlands. 4. d Division of Rheumatology , University of Michigan Scleroderma Program , Ann Arbor , MI , USA. 5. e Division of Rheumatology, Department of Medicine, Jewish General Hospital , McGill University , Montreal , QC , Canada. 6. f Department of Rheumatology AVC, Department of BioMedicine, Division of Rheumatology AOUC, Department of Medicine and Denothe Centre , University of Florence , Florence , Italy. 7. g Centre for Rheumatology and Connective Tissue Diseases , Royal Free Hospital , London , UK. 8. h Department of Medicine, Division of Rheumatology and Clinical Immunology , University of Pittsburgh School of Medicine , Pittsburgh , PA , USA. 9. i Department of Rheumatology , University Hospital 12 de Octubre , Madrid , Spain. 10. j Department of Rheumatology , University of Lübeck, Lung Research Center Borstel, a Leibniz institute , Lübeck , Germany. 11. k Department of Internal Medicine 3 and Institute for Clinical Immunology , University of Erlangen-Nuremberg , Erlangen , Germany. 12. l Department of Molecular and Clinical Sciences, Clinical Medicine , University of Marche , Ancona , Italy. 13. m Department of Medicine, Division of Rheumatology, Clinical Immunology and Allergy , Georgetown University School of Medicine , Washington , DC , USA. 14. n Department of Medicine and Dermatology, Division of Immunology and Rheumatology , Stanford University , Stanford , CA , USA. 15. o Department of Medicine, Division of Rheumatology and Immunology , Medical University of South Carolina , Charleston , SC , USA. 16. p Department of Medicine, Division of Rheumatology, Clinical Immunology and Allergy , Northwestern University , Chicago , IL , USA. 17. q Department of Rheumatology and Clinical Immunology, Justus-Liebig University Giessen, Kerckhoff Clinic , Bad Nauheim , Germany. 18. r Department of Rheumatic Diseases , Radboud University Nijmegen Medical Centre , Nijmegen , The Netherlands. 19. s Department of Rheumatology , University of Basel , Basel , Switzerland. 20. t Department of Rheumatology , Lund University Hospital , Lund , Sweden. 21. u Centre for Musculoskeletal Research, Institute of Inflammation and Repair , The University of Manchester, Manchester Academic Health Science Centre , Manchester , UK. 22. v Division of Rheumatology , University of California Los Angeles (UCLA) , Los Angeles , CA , USA. 23. w Department of Rheumatology and Immunology , University of Pécs, Clinical Center , Pécs , Hungary. 24. x Department of Rheumatology and Internal Medicine , Medical University of Bialystok , Białystok , Poland. 25. y Scleroderma Programme, Leeds Institute of Rheumatic and Musculoskeletal Medicine, LMBRU , University of Leeds , Leeds , UK. 26. z Research Laboratory and Academic Division of Clinical Rheumatology , University of Genova, IRCCS AOU S Martino , Genova , Italy. 27. aa Department of Dermatology , University of Cologne , Cologne , Germany. 28. ab Inflammatory Bowel Disease Unit , Royal Free London NHS Foundation Trust , London , UK. 29. ac Hamburg Center for Paediatric Rheumatology , Eilbek Clinic , Hamburg , Germany. 30. ad Department of Internal Medicine , Paris Descartes University, the Public Hospitals of Paris , Paris , France. 31. ae Institute of Rheumatology , University of Belgrade School of Medicine , Belgrade , Serbia. 32. af Division of Rheumatology, Department of Internal Medicine , University of Toledo Medical Center , Toledo , OH , USA. 33. ag Division of Rheumatology, Department of Internal Medicine, School of Medicine , University of Utah , Salt Lake City , UT , USA. 34. ah University of Texas Health Science Center at Houston , Houston , TX , USA. 35. ai New Orleans Scleroderma and Sarcoidosis Patient Care and Research Center , Tulane University Lung Center , New Orleans , LA , USA. 36. aj Division of Rheumatology, Department of Medicine, St Joseph Health Care , University of Western Ontario , London , ON , Canada.
Abstract
OBJECTIVES: Systemic sclerosis (SSc) is heterogenous. The objectives of this study were to evaluate the purpose, strengths and limitations of existing SSc subset criteria, and identify ideas among experts about subsets. METHODS: We conducted semi-structured interviews with randomly sampled international SSc experts. The interview transcripts underwent an iterative process with text deconstructed to single thought units until a saturated conceptual framework with coding was achieved and respondent occurrence tabulated. Serial cross-referential analyses of clusters were developed. RESULTS: Thirty experts from 13 countries were included; 67% were male, 63% were from Europe and 37% from North America; median experience of 22.5 years, with a median of 55 new SSc patients annually. Three thematic clusters regarding subsetting were identified: research and communication; management; and prognosis (prediction of internal organ involvement, survival). The strength of the limited/diffuse system was its ease of use, however 10% stated this system had marginal value. Shortcomings of the diffuse/limited classification were the risk of misclassification, predictions/generalizations did not always hold true, and that the elbow or knee threshold was arbitrary. Eighty-seven percent use more than 2 subsets including: SSc sine scleroderma, overlap conditions, antibody-determined subsets, speed of progression, and age of onset (juvenile, elderly). CONCLUSIONS: We have synthesized an international view of the construct of SSc subsets in the modern era. We found a number of factors underlying the construct of SSc subsets. Considerations for the next phase include rate of change and hierarchal clustering (e.g. limited/diffuse, then by antibodies).
OBJECTIVES:Systemic sclerosis (SSc) is heterogenous. The objectives of this study were to evaluate the purpose, strengths and limitations of existing SSc subset criteria, and identify ideas among experts about subsets. METHODS: We conducted semi-structured interviews with randomly sampled international SSc experts. The interview transcripts underwent an iterative process with text deconstructed to single thought units until a saturated conceptual framework with coding was achieved and respondent occurrence tabulated. Serial cross-referential analyses of clusters were developed. RESULTS: Thirty experts from 13 countries were included; 67% were male, 63% were from Europe and 37% from North America; median experience of 22.5 years, with a median of 55 new SSc patients annually. Three thematic clusters regarding subsetting were identified: research and communication; management; and prognosis (prediction of internal organ involvement, survival). The strength of the limited/diffuse system was its ease of use, however 10% stated this system had marginal value. Shortcomings of the diffuse/limited classification were the risk of misclassification, predictions/generalizations did not always hold true, and that the elbow or knee threshold was arbitrary. Eighty-seven percent use more than 2 subsets including: SSc sine scleroderma, overlap conditions, antibody-determined subsets, speed of progression, and age of onset (juvenile, elderly). CONCLUSIONS: We have synthesized an international view of the construct of SSc subsets in the modern era. We found a number of factors underlying the construct of SSc subsets. Considerations for the next phase include rate of change and hierarchal clustering (e.g. limited/diffuse, then by antibodies).
Authors: Gabriela Schmajuk; Bimba F Hoyer; Martin Aringer; Sindhu R Johnson; David I Daikh; Thomas Dörner Journal: Arthritis Care Res (Hoboken) Date: 2018-09-11 Impact factor: 4.794
Authors: Vincent Sobanski; Jonathan Giovannelli; Yannick Allanore; Gabriela Riemekasten; Paolo Airò; Serena Vettori; Franco Cozzi; Oliver Distler; Marco Matucci-Cerinic; Christopher Denton; David Launay; Eric Hachulla Journal: Arthritis Rheumatol Date: 2019-08-12 Impact factor: 10.995