DRESS syndrome cases in the US: rare, morbid, and costly

Published online: August 31, 2018

Drug Reaction with Eosinophilia and Systemic Symptoms (DRESS) syndrome is a rare drug hypersensitivity reaction that often presents with rash, fever, eosinophilia, and organ injury, typically of the liver or kidney. Eosinophilia refers to an excess of eosinophils, a type of white blood cell often involved in allergic reactions, in the peripheral blood. DRESS syndrome has been caused by many drug classes in the past, most notably the anti-seizure medications and antibiotics. Symptoms of DRESS syndrome can occur days to weeks into taking the drug, and even after a medication is stopped. The associated mortality rate for DRESS syndrome is from 5-10%. Treatment includes stopping the offending drug and supportive care; steroids are often used for treatment as well. Identification of the causative agent is reliant on the patient history and clinicians’ judgement. Although this severe hypersensitivity syndrome was first recognized over 50 years ago, United States (US) data on DRESS syndrome is limited, largely due to challenges identifying cases: it is not a coded entry in the allergy list’s “reaction” field; it does not have a corresponding billing code that clinicians can use when they diagnose DRESS syndrome; and there is no comprehensive registry for DRESS or other severe cutaneous adverse reactions in the US.

In an article recently published in The Journal of Allergy and Clinical Immunology: In Practice, Wolfson et al. used informatics methods on the allergy list text to identify DRESS cases. The informatics methods used allergist-derived key words (eosinophilia, hypersensitivity syndrome, DRESS, drug-induced hypersensitivity syndrome, Anticonvulsant Hypersensitivity Syndrome (AHS), and drug hypersensitivity syndrome). These key words were used to search the free text in the allergy list using natural language processing, which considers common misspellings and negation (for example, no eosinophilia). Possible DRESS cases were then manually reviewed by an allergist/immunologist to determine if they represented true DRESS cases.

Sixty-nine DRESS patients were identified from 538 hypersensitivity reactions triggered by the informatics searching. The 69 cases came from a total population of approximately 3.2 million patients, corresponding to a DRESS syndrome prevalence of 2.18 per 100,000 patients. In the DRESS syndrome cohort, 29 patients had liver injury only, 29 had kidney injury only, and 11 had both liver and kidney injury. Antibiotics were the most common drug culprits (74%), with vancomycin alone responsible for 40% of the cases. Anticonvulsants were also common culprits (29%); however, almost half (45%) of the patients had two or more drugs as potential culprits. Two patients, both with kidney injury (3%) suffered an in-hospital death due to sepsis. DRESS was resource-intensive; 43 (62%) patients were hospitalized specifically for DRESS syndrome treatment. These patients had a median length of hospital stay of 9 days and their cost of care exceeded $17,000 per patient, which is more than the cost of most asthma admissions.

This study describes the largest series of DRESS patients identified in the US to date. The identification of cases used novel free text searching in the electronic health record allergy list, which could be a model for studying DRESS syndrome, and other rare hypersensitivity reactions, throughout the US.

The Journal of Allergy and Clinical Immunology: In Practice is an official journal of the AAAAI, focusing on practical information for the practicing clinician.

18-00328, Drug reaction Eosinophilia and Systemic Symptoms (DRESS) Syndrome Identified in the Electronic Health Record Allergy Module
By Anna R. Wolfson, Li Zhou, Yu Li, Neelam A. Phadke, Ohn A. Chow, Kimberly G. Blumenthal