There Is a Need for New Systemic Sclerosis Subset Criteria: A Content Analytic Approach

Sindhu Johnson¹, Medha Soowamber², Jaap Fransen³, Dinesh Khanna⁴, Frank H.J. van den Hoogen⁵, Murray Baron⁶, Marco Matucci Cerinic⁷, Christopher P. Denton⁸, Thomas A. Medsger Jr.⁹, Patricia E. Carreira¹⁰, Gabriela Riemekasten¹¹, Jorg HW. Distler¹², Armando Gabrielli¹³, Virginia D. Steen¹⁴, Lorinda Chung¹⁵, Richard Silver¹⁶, John Varga¹⁷, Ulf Müller-Ladner¹⁸, Madelon C. Vonk¹⁹, Ulrich A. Walker²⁰, Frank Wollheim²¹, Ariane L. Herrick²², Daniel E. Furst²³, Lazlo Czirjak²⁴, Otylia Kowal-Bielecka²⁵, Francesco Del Galdo²⁶, Maurizio Cutolo²⁷, Nicolas Hunzelmann²⁸, Charles Murray²⁹, Ivan Foeldvari³⁰, Luc Mouthon³¹, Nemanja Damjanov³², Bashar Kahaleh³³, Tracy M. Frech³⁴, Shervin Assassi³⁵, Lesley Ann Saketkoo³⁶ and Janet E. Pope³⁷, ¹Toronto Scleroderma Program, Toronto Western Hospital, Mount Sinai Hospital, University of Toronto, University Health Network Pulmonary Hypertension Programme, Toronto, ON, Canada, ²Rheumatology, University of Toronto/ Toronto Western Hospital, Toronto, ON, Canada, ³Rheumatology, Radboud University Medical Centre, Nijmegen, Netherlands, ⁴Medicine, University of Michigan, Ann Arbor, MI, ⁵Rheumatology, Sint Maartenskliniek, Nijmegen, Netherlands, ⁶Pavillion A, Rm 216, Jewish General Hospital, Montreal, QC, Canada, ⁷Department of BioMedicine, Division of Rheumatology, Transition Unit, University of Florence, Firenze, Italy, ⁸Centre for Rheumatology, Royal Free Hospital, London, United Kingdom, ⁹Medicine/Rheumatology, Univ of Pittsburgh, Pittsburgh, PA, ¹⁰Department of Rheumatology, Hospital Universitario 12 de Octubre, Madrid, Spain, ¹¹Rheumatology, Human medicine, BERLIN, Germany, ¹²Department of Internal Medicine 3, University of Erlangen-Nuremberg, Erlangen, Germany, ¹³Clinica Medica, Università Politecnica delle Marche, Ancona, Italy, ¹⁴Rheumatology, Georgetown University Medical Center, Washington, DC, ¹⁵Division of Immunology and Rheumatology, Stanford University School of Medicine, Stanford, CA, ¹⁶Div Rheumatology & Immunology, Medical University of South Carolina, Charleston, SC, ¹⁷Medicine, Northwestern University Feinberg School of Medicine, Chicago, IL, ¹⁸Rheumatology, Internal Medicine, Bad Nauheim, Germany, ¹⁹Rheumatology, Rheumatology, Nijmegen, Netherlands, ²⁰Rheumatology, Systemic Sclerosis, Basel, Switzerland, ²¹Rheumatology, Lund University Hospital, Lund, Sweden, ²²Centre for Musculoskeletal Research, University of Manchester, Manchester Academic Health Science Centre, Manchester, United Kingdom, ²³Medicine, University of California, Los Angeles, David Geffen School of Medicine, Los Angeles, CA, ²⁴University of Pécs Medical Center, Pécs, Hungary, ²⁵Department of Rheumatology and Internal Medicine, Medical University of Bialystok, Bialystok, Poland, ²⁶University of Leeds, Leeds, United Kingdom, ²⁷Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genova, Genova, Italy, ²⁸Department of Dermatology, University of Cologne, Cologne, Germany, ²⁹Department of Gastroenterology, Royal Free Hospital, London, United Kingdom, ³⁰Rheumatology, Hamburg, Germany, ³¹Department of Internal Medicine, Department of Internal Medicine, Cochin Hospital, Referent Center for Necrotizing Vasculitis and Systemic Sclerosis, Paris-Descartes University, AP-HP, Paris, France, ³²Resavska 69, Institute of Rheumatology, Belgrade, Serbia, ³³Medicine/Rheumatology, The University of Toledo, Toledo, OH, ³⁴Div of Rheumatology, University of Utah Medical Ctr, Salt Lake City, UT, ³⁵Rheumatology, University of Texas Medical School at Houston, Houston, TX, ³⁶Tulane University Lung Center, New Orleans Scleroderma and Sarcoidosis Patient Care and Research Center, New Orleans, LA, ³⁷University of Western Ontario, London, ON, Canada

Meeting: 2015 ACR/ARHP Annual Meeting

Date of first publication: September 29, 2015

Keywords: Arthritis, fibrosis, Rheumatic disease, scleroderma and systemic sclerosis

Session Information

Date: Monday, November 9, 2015

Title: Systemic Sclerosis, Fibrosing Syndromes and Raynaud's - Clinical Aspects and Therapeutics Poster II

Session Type: ACR Poster Session B

Session Time: 9:00AM-11:00AM

Background/Purpose: 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 measured but is considered to be real. To evaluate the purpose, strengths and limitations of the limited/diffuse subset criteria, and identify areas requiring improvement.

Methods: We conducted a content analytic study consisting of semi-structured interviews with 30 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. 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).

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

Disclosure: S. Johnson, None; M. Soowamber, None; J. Fransen, None; D. Khanna, Bristol-Myers Squibb, 2,EMD Serono, 2,Genentech and Biogen IDEC Inc., 2,Bayer, 5,Biogen Idec, 5,Cytori, 5,EMD Serono, 5,Forward, 5,Genentech and Biogen IDEC Inc., 5,Gilead, 5,Lycera, 5,Seattle Genetics, 5; F. H. J. van den Hoogen, None; M. Baron, None; M. Matucci Cerinic, None; C. P. Denton, None; T. A. Medsger Jr., None; P. E. Carreira, None; G. Riemekasten, None; J. H. Distler, None; A. Gabrielli, None; V. D. Steen, None; L. Chung, Gilead, 4; R. Silver, None; J. Varga, None; U. Müller-Ladner, None; M. C. Vonk, None; U. A. Walker, None; F. Wollheim, None; A. L. Herrick, None; D. E. Furst, Gilead, 2,GlaxoSmithKline, 2,NIH, 2,Novartis Pharmaceutical Corporation, 2,Pfizer Inc, 2,Roche Pharmaceuticals, 2,Genentech and Biogen IDEC Inc., 2,UCB, 2,Abbvie, 5,Actelion Pharmaceuticals US, 5,Amgen, 5,Bristol-Myers Squibb, 5,Cytori, 5,Janssen Pharmaceutica Product, L.P., 5,Gilead, 5,GlaxoSmithKline, 5,NIH, 5,Novartis Pharmaceutical Corporation, 5,Pfizer Inc, 5,Roche Pharmaceuticals, 5,Genentech and Biogen IDEC Inc., 5,UCB, 5,Abbvie, 8,Actelion Pharmaceuticals US, 8,Bristol-Myers Squibb, 2,Amgen, 2,Actelion Pharmaceuticals US, 2,Abbvie, 2,UCB, 8; L. Czirjak, None; O. Kowal-Bielecka, None; F. Del Galdo, None; M. Cutolo, None; N. Hunzelmann, None; C. Murray, None; I. Foeldvari, None; L. Mouthon, None; N. Damjanov, None; B. Kahaleh, None; T. M. Frech, None; S. Assassi, None; L. A. Saketkoo, None; J. E. Pope, Amgen, 2.

To cite this abstract in AMA style:

Johnson S, Soowamber M, Fransen J, Khanna D, van den Hoogen FHJ, Baron M, Matucci Cerinic M, Denton CP, Medsger TA Jr., Carreira PE, Riemekasten G, Distler JH, Gabrielli A, Steen VD, Chung L, Silver R, Varga J, Müller-Ladner U, Vonk MC, Walker UA, Wollheim F, Herrick AL, Furst DE, Czirjak L, Kowal-Bielecka O, Del Galdo F, Cutolo M, Hunzelmann N, Murray C, Foeldvari I, Mouthon L, Damjanov N, Kahaleh B, Frech TM, Assassi S, Saketkoo LA, Pope JE. There Is a Need for New Systemic Sclerosis Subset Criteria: A Content Analytic Approach [abstract]. Arthritis Rheumatol. 2015; 67 (suppl 10). https://acrabstracts.org/abstract/there-is-a-need-for-new-systemic-sclerosis-subset-criteria-a-content-analytic-approach/. Accessed .

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