Current expert consensus for the treatment of adult patients with drug reaction with eosinophilia and systemic symptoms

1. A large majority of experts in this study came to a unified consensus on how to approach the diagnosis and treatment of patients with drug reaction with eosinophilia and systemic symptoms (DRESS).

2. The current consensus for DRESS can inform the diagnostic workup, severity grading, and treatment approaches.

Evidence Rating Level: 5 (Poor)

Study Rundown: Drug reaction with eosinophilia and systemic symptoms (DRESS) is a hypersensitivity reaction characterized by infiltrated maculopapular exanthema, facial edema, lymphadenopathy, organ damage, fever, and eosinophilia. The severity can be mild or lead to severe life-threatening effects; however, no broadly accepted DRESS severity scoring system exists. Furthermore, there is no consensus about the approach to corticosteroid dosing tapering for patients suffering from DRESS. Therefore, this study focused on identifying an expert consensus on DRESS diagnostic workup, severity grading, and treatment. In the first round, 82 of 100 statements reached a consensus from 54 experts. At the end of the second round, 93 statements agreed on basic diagnostic workup procedures, severity- and organ-specific investigations, and severity assessments. Limitations included limited information on DRESS due to its low prevalence, lack of ethnicity and region-associated differences, the broad application of the investigations and results, and the need for more diverse specialists within the panel (80% dermatologists).

Click to read the study in JAMA Dermatology

Relevant Reading: Spanish Guidelines for Diagnosis, Management, Treatment, and Prevention of DRESS Syndrome

In-Depth [expert opinion]: A Delphi approach was employed to determine expert consensus on DRESS diagnostic workup, severity grading, and treatment. Four experts were in charge of guiding the Delphi process by reviewing the literature and drafting initial statements. The literature search included original articles, case reports/series, meta-analyses, clinical trials, and open studies published between 2002 and 2022 across various databases (i.e., PubMed, Embase, CINAHL, the Cochrane Library, and ClinicalTrials.gov). Suggested statements were updated based on the results and comments across two Delphi rounds. Then, experts were identified based on their publication record and/or participation in DRESS expert networks and initiatives. In total, 57 experts (dermatologists and allergologists) from 21 countries across four continents were invited via email to participate in the Delphi consensus; of the 57, 54 participated in the survey between July and September 2022. The first round consisted of 100 statements regarding diagnoses, workup, severity grading, and management. The severity assessment was based on Japanese and French severity criteria and the Sepsis-Related Organ Failure Assessment score values. The level of agreeance of statements was graded on a scale of 1 (extremely inappropriate) to 9 (extremely appropriate). In the second round, experts rated 13 statements: 11 were revisions of the uncertain statements from round one, and 2 were additional statements within the diagnosis and allergological workup. The degree of agreement was calculated using the RAND-UCLA Appropriateness Method, and consensus was reached when the median appropriateness was valued at seven or higher with a disagreement index lower than one. In total, 93 statements achieved consensus, with experts agreeing on basic diagnostic workup procedures, severity-specific investigations, organ-specific investigations and severity assessments. A severity assessment consensus was reached for the extent of liver, kidney, blood involvement, and the damage to other organs. General recommendations were also generated from allergological workups and the post-acute phase follow-up of patients with DRESS.

Image: PD

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