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

S. R. Johnson*, M. L. Soowamber, J. Fransen, D. Khanna, F. Van Den Hoogen, M. Baron, M. Matucci-Cerinic, C. P. Denton, T. A. Medsger, P. E. Carreira, G. Riemekasten, J. Distler, A. Gabrielli, V. Steen, L. Chung, R. Silver, J. Varga, U. Müller-Ladner, M. C. Vonk, U. A. WalkerF. A. Wollheim, A. Herrick, D. E. Furst, L. Czirjak, O. Kowal-Bielecka, F. Del Galdo, M. Cutolo, N. Hunzelmann, C. D. Murray, I. Foeldvari, L. Mouthon, N. Damjanov, B. Kahaleh, T. Frech, S. Assassi, L. A. Saketkoo, J. E. Pope

*Corresponding author for this work
11 Citations (Scopus)


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).

Original languageEnglish
JournalScandinavian Journal of Rheumatology
Issue number1
Pages (from-to)62-70
Number of pages9
Publication statusPublished - 02.01.2018

Research Areas and Centers

  • Academic Focus: Center for Infection and Inflammation Research (ZIEL)


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