New ACR EULAR Guidelines for Systemic Sclerosis Classification

  • Sindhu R. Johnson
Scleroderma (J Varga, Section Editor)
Part of the following topical collections:
  1. Topical Collection on Scleroderma


The American College of Rheumatology and European League Against Rheumatism classification criteria for systemic sclerosis are a significant advancement in the field. This article describes the innovative, rigorous, criteria development strategy that was used. The new criteria build upon previous criteria by incorporating important elements (proximal scleroderma, sclerodactyly, digital pits, pulmonary fibrosis, Raynaud’s phenomenon, and scleroderma specific autoantibodies). The new criteria add emphasis to the vasculopathic manifestations, and include the early manifestation of puffy fingers. Together, these enhancements have resulted in a shift in the conceptual framework of the disease for the next generation. The new criteria have improved sensitivity and specifically, particularly among cases with early disease, mild disease, or limited disease. The ability to classify more cases, at an earlier stage, may confer the opportunity to intervene and prevent disease progression. Undoubtedly, this will lead to a paradigm shift in the conduct of clinical trials in systemic sclerosis.


Systemic sclerosis Scleroderma Classification criteria Classification Misclassification Clinical epidemiology Cohort Delphi Nominal group technique Bayesian Multicriteria decision analysis Conjoint analysis Forced choice 1000Minds Mixed connective tissue disease 



Sindhu Johnson has been awarded a Canadian Institutes of Health Research Clinician Scientist Award and is supported by the Freda Fejer Fund and the Norton-Evans Fund for Scleroderma Research.

Compliance with Ethics Guidelines

Conflict of Interest

Sindhu Johnson declares grants from American College of Rheumatology and from the European League Against Rheumatism, during the writing of this paper. She declares that she is Co-chair of the American College of Rheumatology Classification and Response Criteria subcommittee.

Human and Animal Rights and Informed Consent

This article does not contain any studies with human or animal subjects performed by any of the authors.


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Copyright information

© Springer Science+Business Media New York 2015

Authors and Affiliations

  1. 1.Toronto Scleroderma Program, Mount Sinai Hospital, Toronto Western Hospital, Division of Rheumatology, Department of MedicineUniversity of TorontoTorontoCanada
  2. 2.Institute of Health Policy, Management and EvaluationUniversity of TorontoTorontoCanada

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