Article Text

AB0727 There is a Need for New Systemic Sclerosis Subset Criteria. A Content Analytic Approach
  1. S.R. Johnson1,
  2. J. Fransen2,
  3. D. Khanna3,
  4. F. van den Hoogen2,
  5. M. Baron4,
  6. M. Matucci-Cerinic5,
  7. C. Denton6,
  8. T.A. Medsger7,
  9. P. Carreira8,
  10. G. Riemekasten9,
  11. J. Distler10,
  12. A. Gabrielli11,
  13. V. Steen12,
  14. L. Chung13,
  15. R. Silver14,
  16. J. Varga15,
  17. U. Muller-Ladner16,
  18. M. Vonk2,
  19. U. Walker17,
  20. F. Wollheim18,
  21. A. Herrick19,
  22. D. Furst20,
  23. L. Czirjak21,
  24. O. Kowal-Bielecka22,
  25. F. DelGaldo23,
  26. M. Cutolo24,
  27. N. Hunzelmann25,
  28. C. Murray6,
  29. I. Foeldvari26,
  30. L. Mouthon27,
  31. N. Damjanov28,
  32. B. Kahaleh29,
  33. T. Frech30,
  34. S. Assassi31,
  35. L.A. Saketkoo32,
  36. J. Pope33
  1. 1University of Toronto, Toronto, Canada
  2. 2Radboud University, Nijmegen, Netherlands
  3. 3University of Michigan, Ann Arbor, United States
  4. 4Jewish General Hospital, Montreal, Canada
  5. 5University of Florence, Firenze, Italy
  6. 6Royal Free Hospital, London, United Kingdom
  7. 7University of Pittsburgh, Pittsburgh, United States
  8. 8Hospital Universitario 12 de Octubre, Madrid, Spain
  9. 9Leibniz Institute, Berlin
  10. 10University of Erlangen-Nuremberg, Erlangen, Germany
  11. 11Università Politecnica delle Marche, Ancona, Italy
  12. 12Georgetown University, Georgetown
  13. 13Stanford University, Standford
  14. 14Medical University of South Carolina, Charleston
  15. 15Northwestern University, Chicago, United States
  16. 16Justus-Liebig University Giessen, Bad Nauheim, Germany
  17. 17University of Basel, Basel, Switzerland
  18. 18Lund University, Lund, Sweden
  19. 19University of Manchester, Manchester, United Kingdom
  20. 20UCLA, Los Angeles, United States
  21. 21University of Pécs, Pecs, Hungary
  22. 22Medical University of Bialystok, Bialystok, Poland
  23. 23University of Leeds, Leeds, United Kingdom
  24. 24University of Genova, Genova, Italy
  25. 25University of Cologne, Cologne
  26. 26An der Schön Klinik Hamburg Eilbe, Hamburg, Germany
  27. 27Hôpital Cochin, Paris, France
  28. 28Belgrade University, Belgrade, Serbia
  29. 29University of Toledo, Toledo
  30. 30University of Utah, Salt Lake City
  31. 31University of Texas, Houston
  32. 32Tulane University, New Orleans, United States
  33. 33Western University, London, Canada


Background Systemic sclerosis (SSc) is a family of diseases unified by the presence of immune activation, vasculopathy and fibrosis. The concept of SSc subsets cannot be easily defined but is clinically indisputable.

Objectives To evaluate the purpose, strengths and limitations of the limited/diffuse subset criteria, and identify areas requiring improvement.

Methods We conducted a cross-sectional study with 30 SSc experts using a semi-structured interview. 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. This was followed by serial cross-referential analyses establishing a set of pervasive complex thought clusters.

Results Of the 30 experts, 26 (87%) were male, 19 (63%) were from Europe and 11 (37%) were from North America. The experts had seen SSc patients for a mean 23 (SD 10.7) years, and saw a mean of 122 (SD 185) new SSc patients annually. Three thematic clusters were noted regarding the utility of subsetting: to facilitate research and communication, to inform management, and to inform prognosis (prediction of internal organ involvement, survival). The strength of the limited/diffuse system was its ease of use, however 10% stated this system has “little or no value”. Limitations of the diffuse/limited classification were the risk of misclassification, predictions/generalizations did not always hold true, and that the elbow or knee threshold is arbitrary. 87% use more than 2 subsets including: SSc sine scleroderma, overlap conditions, antibody determined subsets, subsetting based on speed of progression, and age of onset (juvenile, elderly). Considerations for the next phase of criteria development include incorporation of rate of change and hierarchal clustering (limited/diffuse, then by antibodies).

Conclusions We interviewed international SSc experts and synthesized their views on subset criteria. These results can inform future efforts to develop revised criteria to guide research, prognostication and management.

Acknowledgements This work was supported by the World Scleroderma Foundation.

Disclosure of Interest S. Johnson Grant/research support from: Canadian Institutes of Health Research, J. Fransen: None declared, D. Khanna Grant/research support from: NIH/NIAMS K24, F. van den Hoogen: None declared, M. Baron: None declared, M. Matucci-Cerinic: None declared, C. Denton: None declared, T. Medsger: None declared, P. Carreira: None declared, G. Riemekasten: None declared, J. Distler: None declared, A. Gabrielli: None declared, V. Steen: None declared, L. Chung: None declared, R. Silver: None declared, J. Varga: None declared, U. Muller-Ladner Grant/research support from: EULAR/EUSTAR and FP7 Desscipher, M. Vonk: None declared, U. Walker: None declared, F. Wollheim: None declared, A. Herrick: None declared, D. Furst: None declared, L. Czirjak: None declared, O. Kowal-Bielecka: None declared, F. DelGaldo: None declared, M. Cutolo: None declared, N. Hunzelmann: None declared, C. Murray: None declared, I. Foeldvari: None declared, L. Mouthon: None declared, N. Damjanov: None declared, B. Kahaleh: None declared, T. Frech: None declared, S. Assassi: None declared, L. A. Saketkoo: None declared, J. Pope: None declared

Statistics from

Request permissions

If you wish to reuse any or all of this article please use the link below which will take you to the Copyright Clearance Center’s RightsLink service. You will be able to get a quick price and instant permission to reuse the content in many different ways.