DECLARATIONS ETHICAL APPROVAL Patients were enrolled following written informed consent. Ethical approval was granted by the Institutional Review Board (IRB) at UCSD. CONSENT FOR PUBLICATION: N/A AVAILABILITY OF DATA AND MATERIALS All data generated or analyzed during this study are included in this published article COMPETING INTERESTS Roxana Coras declares that she has no conflict of interest. Arthur Kavanaugh declares that he has no conflict of interest. Tristan Boyd declares that he has no conflict of interest. Quyen Huynh declares that she has no conflict of interest. Brian Pedersen declares that he has no conflict of interest. Aaron M Armando declares that he has no conflict of interest. Signe Dahlberg-Wright declares that she has no conflict of interest. Sara Marsal declares that she has no conflict of interest. Mohit Jain declares that he has no conflict of interest. Taraneh Paravar declares that she has no conflict of interest. Oswald Quehenberger declares that he has no conflict of interest. Monica Guma declares that she has no conflict of interest. The authors report no conflict of interest.
The classification of thalidomide as an orphan drug with anti-inflammatory actions has led to its off-label use in conditions refractory to other medications. Although the observed clinical effects of thalidomide suggest it to have immunomodulatory capabilities, the mechanism of action is unclear. Here we review both the positive and negative studies of thalidomide at the bench in order to improve our understanding of the possible mechanisms of this drug in treating a variety of diseases at the bedside. Studies on the effects of thalidomide on the innate and adaptive immune system as well as tumorigenesis and angiogenesis are discussed.
Summary
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.
scite is a Brooklyn-based organization that helps researchers better discover and understand research articles through Smart Citations–citations that display the context of the citation and describe whether the article provides supporting or contrasting evidence. scite is used by students and researchers from around the world and is funded in part by the National Science Foundation and the National Institute on Drug Abuse of the National Institutes of Health.