Clinico-epidemiological Analysis Of Patients With Diagnosis Of Drug Reaction With Eosinophilia And Systemic Symptoms (DRESS) In Ruijin Hospital,Shanghai: Retrospective Study Of 37 Patients

Backgrounds:Drug reaction with eosinophilia and systemic symptoms(DRESS)syndrome or also known as drug-induced hypersensitivity syndrome(DIHS)is a rare but severe systemic adverse drug reaction usually caused by administration of specific drug.Many reports regarding autoimmune complications following the remission of DRESS had been reported.The present study sought to analyze the clinical characteristics,treatment and autoimmune sequelae observed in patients admitted to Ruijin hospital,Shanghai.Methods and Results:We retrospectively analyzed all patients admitted to the dermatology ward of Ruijin hospital,Shanghai from January 2006 to July 2015 with the diagnosis of DRESS as per the diagnostic criteria proposed by Bocquet et al were included in this study.The clinical manifestations observed in the study population were studied and the common offending drugs were identified.Treatment regimes and outcomes as well as post remission autoimmune sequelae following DRESS syndrome were also analyzed.The data of this study were expressed as means(ranges),correlations were analyzed using Spearman’s coefficients,comparisons were performed with a Mann-Whitney U-test.The SPSS for Windows version 18.0(SPSS Inc.,Chicago,IL,USA)program was used for all analyses.A P value of <0.05 was considered to indicate statistical significance.Thirty-seven cases of DRESS(22 males and 15 females;Age range 18 to 91 years,average age,48.62±20 years)were included in this study.The most common causative drugs were Allopurinol(12 patients,32.4 %),SASP(11 patients,29.7%),Carbamazepine(6 patients,16.2%),followed by Mexiletine(2 patients,5.4%),Amoxycilline(1 patient,2.7%),Azithromycin(1 patient,2.7%),Mesalazine(1 patient,2.7%),Rifampicin(1 patient,2.7%),Sparfloxacin(1 patient,2.7%)and Vancomycin(1 patient,2.7%).The average intervals from the introduction of the causative agent to the onset of clinical manifestations ranged from 2 to 90 days(average 29.5±17.5 days).High white blood cell count was observed in 94.6%(35 out of 37 patients)of the study population,whilst peripheral blood eosinophilia was observed in 89.2% of the patients(33 out of 37 patients).Liver was the most common organ involvement in DRESS syndrome of our study population,which was seen in 75.7%(28/37).Lymphadenopathy was positive in 42.2%(16 out of 37 patients).Renal involvement and hepatosplenomegaly were also involved in some cases.All cases except for 3 cases were treated systemically with corticosteroids.Intravenous immunoglobulin therapy was combined with steroid therapy in 18 patients(48.6 %).Follow up was completed in 23 of the patients and several autoimmune sequelae were observed in this study,such as diabetes,vitiligo and alopecia areata.Some patients were loss to follow up.Conclusions : Prompt culprit drug withdrawal,optimal dosing of systemic corticosteroids and intravenous immunoglobulin administration are associated with good outcome in all study subjects.The incidence of autoimmune sequelae post remission of DRESS syndrome is increasing.Therefore,long term follow up of all DRESS syndrome patients are necessary.s Fas L represents a useful biomarker that can predict the subsequent regression or progression of this syndrome in our study subjects.