There is a need for new systemic sclerosis subset criteria. A content analytic approach

Forskningsoutput: TidskriftsbidragArtikel i vetenskaplig tidskrift


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).


  • S Johnson
  • M. Soowamber
  • J. Fransen
  • Dinesh Khanna
  • F. H. J. van den Hoogen
  • M A Baron
  • M. Matucci-Cerinic
  • C. P. Denton
  • T. Medsger
  • P. Carreira
  • G. Riemekasten
  • J. H. W. Distler
  • A Gabrielli
  • Virginia Steen
  • Lorinda Chung
  • R Silver
  • John Varga
  • Ulf Müller-Ladner
  • M. Vonk
  • Ulrich Walker
  • A. Herrick
  • Daniel E Furst
  • L. Czirjak
  • Otylia Kowal-Bielecka
  • Francesco Del Galdo
  • M Cutolo
  • N. Hunzelmann
  • C. Murray
  • Ivan Foeldvari
  • Luc Mouthon
  • N Damjanov
  • Bashar Kahaleh
  • Tracy M Frech
  • S. Assassi
  • L. Saketkoo
  • J Pope
Externa organisationer
  • University of Toronto
  • Radboud University Nijmegen
  • University of Michigan
  • McGill University
  • University of Florence
  • Royal Free Hospital
  • University of Pittsburgh
  • 12 de Octubre University Hospital
  • British Columbia Children's Hospital
  • Friedrich-Alexander University Erlangen-Nürnberg
  • Marche Polytechnic University
  • Stanford University
  • Northwestern University
  • Justus Liebig University Giessen
  • University Hospital Basel
  • Skåne University Hospital
  • University of Manchester
  • University of California, Los Angeles
  • University of Pécs
  • Medical University of Bialystok
  • University of Leeds
  • Azienda Ospedaliera Universitaria San Martino IST
  • University of Cologne
  • Great Ormond Street Hospital for Children NHS Foundation Trust
  • Paris Descartes University
  • University of Utah
  • University of Texas
  • Tulane University
  • University of Western Ontario

Ämnesklassifikation (UKÄ) – OBLIGATORISK

  • Reumatologi och inflammation
  • Dermatologi och venereologi
Sidor (från-till)62-70
TidskriftScandinavian Journal of Rheumatology
Utgåva nummer1
StatusPublished - 2018
Peer review utfördJa
Externt publiceradJa