We present here the recommendations resulting from this Delphi process. This international consensus includes intravenous CD20 inhibitors as a first line therapy option for moderate to severe pemphigus.

Increased skin IL-31 may play a role in DM-associated itch, and ongoing trials will evaluate the effects of systemic treatment on IL-31 and itch in DM.

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.

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BackgroundEffective treatment options are limited for refractory skin disease in dermatomyositis (DM). Lenabasum is a non-immunosuppressive, synthetic, oral preferential CB2 agonist that triggers resolution of innate immune responses and reduces cytokine production by PBMC from DM patients.ObjectivesThe purpose of this study was to test safety and efficacy of lenabasum (aka JBT-101, anabasum) in DM subjects with refractory, moderate-to-severely active skin disease.MethodsA double-blind, randomised placebo-controlled 16 week Phase 2 trial (JBT101-DM-001; NCT02466243) enrolled adults with documented DM and a Cutaneous Dermatomyositis Disease Area and Severity Index (CDASI) activity score ≥14, minimal active muscle involvement and failure or intolerance to hydroxychloroquine and stable DM medications including immunosuppressants. Subjects received 2 escalating dose levels of lenabasum (20 mg QD X 4 weeks, then 20 mg BID X 8 weeks) or PBO X 12 weeks. Subjects were followed off study drug X 4 weeks. Safety and efficacy outcomes were assessed from Day 1 through end of study at Week 16. The primary efficacy objective was to assess efficacy of lenabasum using CDASI activity score.Results11 adults each received lenabasum and PBO (n=22). Demographic and disease characteristics were similar in both cohorts. Both cohorts had mean CDASI activity scores in the severe range33–35 despite immunosuppressants (19/22 subjects). Lenabasum subjects had clinically meaningful improvement in CDASI activity scores with mean reduction ≥5 points at all visits after 4 weeks. Improvement had statistical significance at end of study (figure 1, p=0.02, 2-sided MMRM) that first became apparent after 4 weeks. Lenabasum provided greater improvement than placebo in CDASI damage index, patient-reported global skin disease and overall disease assessments, skin symptoms including photosensitivity and itch, fatigue, sleep, pain interference with activities, pain, and physical function (examples in figure 1). Improvements in secondary efficacy outcomes reached statistical significance (p≤0.1, 1-sided MMRM) at multiple visits after week 4 (figure 1). There were no serious, severe or unexpected adverse events (AEs) related to lenabasum. Tolerability of lenabasum was excellent with no study drop-outs. Subjects in the lenabasum cohort had numerically more mild AEs than placebo subjects (29 vs. 19) and fewer moderate AEs (4 vs. 7). AEs in ≥3 subjects in any cohort were diarrhoea, dizziness (lightheadedness), fatigue and dry mouth.Abstract FRI0470 – Figure 1Effects of Lenabasum on Efficacy Outcomes in Refractory, Skin-Predominant DMConclusionsLenabasum demonstrated consistent evidence of clinical benefit across multiple efficacy outcomes and had acceptable safety and tolerability in this Phase 2 trial in refractory skin disease in DM. Further evaluation of lenabasum in the treatment of DM is warranted.Disclosure of InterestV. Werth Consultant for: Corbus Pharmaceuticals, Inc., E. Hejazi: None declared, S. Pena: None declared, J. Haber: None declared, J. Okawa:...

Implementation of a short in-office survey immediately after patient care can provide better feedback that can be used to ensure that improvement measures are aimed at making significant strides in improving overall health outcomes for patients.

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