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. 2020 Feb;182(2):410-417.
doi: 10.1111/bjd.18096. Epub 2019 Sep 29.

Developing classification criteria for skin-predominant dermatomyositis: the Delphi process

J S S Concha  1   2 , S Pena  1   2 , R G Gaffney  1   2 , B Patel  1   2 , M Tarazi  1   2 , C J Kushner  1   2 , J F Merola  3 , D Fiorentino  4 , J P Dutz  5 , M Goodfield  6 , F Nyberg  7 , B Volc-Platzer  8 , M Fujimoto  9 , C C Ang  10 , V P Werth  1   2 ; Skin Myositis Delphi Group
Collaborators, Affiliations
Free article

Developing classification criteria for skin-predominant dermatomyositis: the Delphi process

J S S Concha et al. Br J Dermatol. 2020 Feb.
Free article

Abstract

Background: The European League Against Rheumatism/American College of Rheumatology classification criteria for inflammatory myopathies are able to classify patients with skin-predominant dermatomyositis (DM). However, approximately 25% of patients with skin-predominant DM do not meet two of the three hallmark skin signs and fail to meet the criteria.

Objectives: To develop a set of skin-focused classification criteria that will distinguish cutaneous DM from mimickers and allow a more inclusive definition of skin-predominant disease.

Methods: An extensive literature review was done to generate items for the Delphi process. Items were grouped into categories of distribution, morphology, symptoms, antibodies, histology and contextual factors. Using REDCap™, participants rated these items in terms of appropriateness and distinguishing ability from mimickers. The relevance score ranged from 1 to 100, and the median score determined a rank-ordered list. A prespecified median score cut-off was decided by the steering committee and the participants. There was a pre-Delphi and two rounds of actual Delphi.

Results: There were 50 participating dermatologists and rheumatologists from North America, South America, Europe and Asia. After a cut-off score of 70 during the first round, 37 of the initial 54 items were retained and carried over to the next round. The cut-off was raised to 80 during round two and a list of 25 items was generated.

Conclusions: This project is a key step in the development of prospectively validated classification criteria that will create a more inclusive population of patients with DM for clinical research. What's already known about this topic? Proper classification of patients with skin-predominant dermatomyositis (DM) is indispensable in the appropriate conduct of clinical/translational research in the field. The only validated European League Against Rheumatism/American College of Rheumatology criteria for idiopathic inflammatory myopathies are able to classify skin-predominant DM. However, a quarter of amyopathic patients still fail the criteria and does not meet the disease classification. What does this study add? A list of 25 potential criteria divided into categories of distribution, morphology, symptomatology, pathology and contextual factors has been generated after several rounds of consensus exercise among experts in the field of DM. This Delphi project is a prerequisite to the development of a validated classification criteria set for skin-predominant DM.

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References

    1. Da Silva DM, Patel B, Werth VP. Dermatomyositis: a diagnostic dilemma. J Am Acad Dermatol 2018; 79:371-3.
    1. Lundberg IE, Miller FW, Tjarnlund A et al. Diagnosis and classification of idiopathic inflammatory myopathies. J Intern Med 2016; 280:39-51.
    1. Lundberg IE, de Visser M, Werth VP. Classification of myositis. Nat Rev Rheumatol 2018; 14:269-78.
    1. Concha J, Tarazi M, Kushner C et al. The diagnosis and classification of amyopathic dermatomyositis: a historical review and assessment of existing criteria. Br J Dermatol 2019; 180:1001-8.
    1. Ghazi E, Sontheimer RD, Werth VP. The importance of including amyopathic dermatomyositis in the idiopathic inflammatory myositis spectrum. Clin Exp Rheumatol 2013; 31:128-34.

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