Efficacy of add‐on therapy with intravenous immunoglobulin in steroid hyporesponsive DRESS syndrome

Abstract Drug reaction with eosinophilia and systemic symptoms (DRESS) syndrome is a rare, potentially life‐threatening, delayed, drug‐induced hypersensitivity reaction. Immediate withdrawal of the culprit drug and administration of systemic corticosteroids is the most widely accepted treatment. How...

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Main Authors: Da Woon Sim, Jieun Yu, Young‐Il Koh
Format: Article
Language:English
Published: Wiley 2022-03-01
Series:Clinical and Translational Science
Online Access:https://doi.org/10.1111/cts.13201
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author Da Woon Sim
Jieun Yu
Young‐Il Koh
author_facet Da Woon Sim
Jieun Yu
Young‐Il Koh
author_sort Da Woon Sim
collection DOAJ
description Abstract Drug reaction with eosinophilia and systemic symptoms (DRESS) syndrome is a rare, potentially life‐threatening, delayed, drug‐induced hypersensitivity reaction. Immediate withdrawal of the culprit drug and administration of systemic corticosteroids is the most widely accepted treatment. However, it is difficult to manage patients with DRESS syndrome who are not responsive to systemic steroids. We studied the efficacy of intravenous immunoglobulins (IVIGs) in patients with DRESS syndrome unresponsive to systemic steroids. We retrospectively reviewed patients with DRESS syndrome who received IVIG in addition to systemic steroids during 2012–2017 and compared the clinical features and course of DRESS syndrome, before and after IVIG treatment. Eighteen DRESS patients (9 men) were included. The most frequent offending drugs were dapsone in five patients, followed by vancomycin in three patients, and carbamazepine in three patients. Rash, fever, lymphadenopathy, atypical lymphocytes, and hepatic involvement were common clinical findings. IVIG treatment was added within a median time of 7 days from the commencement of systemic steroid therapy. After IVIG treatment (total dosage: 1–2 g/kg), the fever resolved within a median time of 1 day (range, 0–3) and liver enzymes improved substantially within a median time of 13 days (range, 0–27). No severe adverse reactions related to IVIG therapy were observed in this study; however, there was one case of mortality. The addition of IVIG in DRESS syndrome in cases refractory to systemic steroid treatment may be helpful in hastening recovery. However, comparative studies using a placebo group are needed.
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spelling doaj.art-e115dfd5f707414297f3fe6532ef6d702022-12-22T01:01:23ZengWileyClinical and Translational Science1752-80541752-80622022-03-0115378278810.1111/cts.13201Efficacy of add‐on therapy with intravenous immunoglobulin in steroid hyporesponsive DRESS syndromeDa Woon Sim0Jieun Yu1Young‐Il Koh2Division of Allergy, Asthma, and Clinical Immunology Department of Internal Medicine Chonnam National University Medical School Chonnam National University Hospital Gwangju KoreaDivision of Allergy, Asthma, and Clinical Immunology Department of Internal Medicine Chonnam National University Medical School Chonnam National University Hospital Gwangju KoreaDivision of Allergy, Asthma, and Clinical Immunology Department of Internal Medicine Chonnam National University Medical School Chonnam National University Hospital Gwangju KoreaAbstract Drug reaction with eosinophilia and systemic symptoms (DRESS) syndrome is a rare, potentially life‐threatening, delayed, drug‐induced hypersensitivity reaction. Immediate withdrawal of the culprit drug and administration of systemic corticosteroids is the most widely accepted treatment. However, it is difficult to manage patients with DRESS syndrome who are not responsive to systemic steroids. We studied the efficacy of intravenous immunoglobulins (IVIGs) in patients with DRESS syndrome unresponsive to systemic steroids. We retrospectively reviewed patients with DRESS syndrome who received IVIG in addition to systemic steroids during 2012–2017 and compared the clinical features and course of DRESS syndrome, before and after IVIG treatment. Eighteen DRESS patients (9 men) were included. The most frequent offending drugs were dapsone in five patients, followed by vancomycin in three patients, and carbamazepine in three patients. Rash, fever, lymphadenopathy, atypical lymphocytes, and hepatic involvement were common clinical findings. IVIG treatment was added within a median time of 7 days from the commencement of systemic steroid therapy. After IVIG treatment (total dosage: 1–2 g/kg), the fever resolved within a median time of 1 day (range, 0–3) and liver enzymes improved substantially within a median time of 13 days (range, 0–27). No severe adverse reactions related to IVIG therapy were observed in this study; however, there was one case of mortality. The addition of IVIG in DRESS syndrome in cases refractory to systemic steroid treatment may be helpful in hastening recovery. However, comparative studies using a placebo group are needed.https://doi.org/10.1111/cts.13201
spellingShingle Da Woon Sim
Jieun Yu
Young‐Il Koh
Efficacy of add‐on therapy with intravenous immunoglobulin in steroid hyporesponsive DRESS syndrome
Clinical and Translational Science
title Efficacy of add‐on therapy with intravenous immunoglobulin in steroid hyporesponsive DRESS syndrome
title_full Efficacy of add‐on therapy with intravenous immunoglobulin in steroid hyporesponsive DRESS syndrome
title_fullStr Efficacy of add‐on therapy with intravenous immunoglobulin in steroid hyporesponsive DRESS syndrome
title_full_unstemmed Efficacy of add‐on therapy with intravenous immunoglobulin in steroid hyporesponsive DRESS syndrome
title_short Efficacy of add‐on therapy with intravenous immunoglobulin in steroid hyporesponsive DRESS syndrome
title_sort efficacy of add on therapy with intravenous immunoglobulin in steroid hyporesponsive dress syndrome
url https://doi.org/10.1111/cts.13201
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AT youngilkoh efficacyofaddontherapywithintravenousimmunoglobulininsteroidhyporesponsivedresssyndrome