Management and treatment outcome of DRESS patients in Europe: An international multicentre retrospective study of 141 cases

Abstract

Background Drug reaction with eosinophilia and systemic symptoms (DRESS) is a rare but potentially fatal drug hypersensitivity reaction. Objective To explore treatment approaches across Europe and their impact on the disease course, as well as prognostic factors and culprit drugs. Methods In this retrospective European multicentric study, we included patients with probable or certain DRESS (RegiSCAR score ≥ 4) between January 2016 and December 2020. Independent associations between clinical parameters and the risk of intensive care unit admission and mortality at three months were assessed using a multivariable‐adjusted logistic regression model. Results A total of 141 patients from 8 tertiary centres were included. Morbilliform exanthem was the most frequent cutaneous manifestation (78.0%). The mean affected body surface area (BSA) was 67%, 42% of the patients presented with erythroderma, and 24.8% had mucosal involvement. Based on systemic involvement, 31.9% of the patients had a severe DRESS. Anticonvulsants (24.1%) and sulphonamides (22.0%) were the most frequent causative agents. In all, 73% of the patients were treated with systemic glucocorticoids, and 25.5% received topical corticosteroids as monotherapy. Few patients received antiviral drugs or anti‐IL5. No patients received intravenous immunoglobulins. The overall mortality was 7.1%. Independent predictors of mortality were older age (≥57.0 years; fully adjusted OR, 9.80; 95% CI, 1.20–79.93; p = 0.033), kidney involvement (fully adjusted OR, 4.70; 95% CI, 1.00–24.12; p = 0.049), and admission in intensive care unit (fully adjusted OR, 8.12; 95% CI, 1.90–34.67; p = 0.005). Relapse of DRESS and delayed autoimmune sequelae occurred in 8.5% and 12.1% of patients, respectively. Conclusions This study underlines the need for diagnostic and prognostic scores/markers as well as for prospective clinical trials of drugs with the potential to reduce mortality and complications of DRESS.

Retrospective study on the association of human herpesvirus reactivation with severe DRESS: A description of blood and skin reactivations

“…There was comparable proportion of liver involvement regardless of the inducing treatment or viral reactivation. Renal involvements were more frequent (46% vs. 37% in literature), but also associated with a poor prognosis in literature 25,26 . We stated more renal involvement in allopurinol‐induced DRESS, as previously described 25 .…”

“…Renal involvements were more frequent (46% vs. 37% in literature), but also associated with a poor prognosis in literature. 25,26 We stated more renal involvement in allopurinol-induced DRESS, as previously described. 25 We also found tendency for more allopurinol-induced DRESS in patients with prior chronic renal insufficiency, in agreement once again with literature.…”

Síndrome de DRESS (Drug Reaction with Eosinophilia and Systemic Symptoms) por Sulfassalazina em paciente em tratamento para Doença Inflamatória Intestinal

“…7 Os sinais clínicos e laboratoriais associados são: febre, linfadenopatia, anormalidades hematológicas: eosinofilia acima de 700 microL, leucocitose, linfocitose com atipia, neutrofilia, monocitose; e anormalidade laboratorial ou clínica envolvendo qualquer órgão visceral. 8 O fígado é o órgão mais comumente afetado pela síndrome. 9 As alterações nos testes de função hepática são geralmente leves e transitórias, com padrão colestático sendo o mais prevalente (37%), seguido do padrão misto (27%).…”

Relevance of Human Herpesvirus 6 Reactivation in Drug Rash With Eosinophilia and Systemic Symptoms—Reply

“…Another challenge are regional differences of viral reactivation. This is illustrated by variability in viral reactivation patterns in patients with DRESS from Japan and Europe, respectively . We look forward to advances in the field to provide more evidence how to handle viral reactivation in DRESS.…”

“…First, in DRESS, reactivation of HHV-6B occurs more frequently (in about 60% of cases) than does reactivation of CMV, Epstein-Barr virus, and HHV-7. 4 Reactivation of HHV-6B in blood is typically detected weeks after the onset of DRESS. 4 For that reason, it has been assumed that reactivation of the virus is not likely a cause of DRESS and that drugs that trigger DRESS also encourage reactivation of the virus.…”