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1.
Front Immunol ; 15: 1415830, 2024.
Artículo en Inglés | MEDLINE | ID: mdl-39091503

RESUMEN

Objective: Severe cutaneous adverse reactions (SCARs) are rare but life-threatening, with antibiotics being the main cause. This retrospective study from a single center was designed to analyze the culprit drugs, clinical features and treatment outcomes of antibiotic-induced SCARs. Methods: We analyzed cases of antibiotic-induced SCARs in a tertiary hospital in China between January 2013 and January 2024, including Steven-Johnson syndrome (SJS) or Stevens-Johnson syndrome-toxic epidermal necrolysis (SJS-TEN) overlap, toxic epidermal necrolysis (TEN), drug reaction with eosinophilia and systemic symptoms (DRESS) and acute generalized exanthematous pustulosis (AGEP). Descriptive analysis of the demographic characteristics, clinical manifestations, treatment and prognosis were carried out. Results: Among 354 cases of SCARs, 63 validated antibiotic-related cases were included. Cephalosporins (31.7%), penicillins (25.4%), and quinolones (19.0%) were the most common triggers for SCARs. Overall, liver (50.8%), lungs (31.7%), and kidneys (23.8%) were the most frequently affected organ in SCARs cases. Eight patients (28.6%) in the SJS/SJS-TEN overlap group and 8 patients (80.0%) in the TEN group received combination therapy of corticosteroids and IVIG. Patients with SCARs caused by penicillins or cephalosporins could receive alternative treatments such as lincomamides, quinolones, and tetracyclines. The mortality rate in the TEN group was the highest at 20.0%, followed by the SJS/SJS-TEN overlap group (7.1%), and no deaths were observed in the DRESS and AGEP groups. Conclusion: The identification of the culprit antibiotics and the application of alternative antibiotic therapies are crucial for the management of antibiotic-induced SCARs. If complicated underlying conditions and complications like advanced age, cancer and pneumonia coexist with SCARs, patients might be more at risk for mortality.


Asunto(s)
Antibacterianos , Humanos , Estudios Retrospectivos , Masculino , Femenino , Antibacterianos/efectos adversos , Persona de Mediana Edad , Adulto , Anciano , Síndrome de Stevens-Johnson/etiología , Síndrome de Stevens-Johnson/mortalidad , Síndrome de Stevens-Johnson/tratamiento farmacológico , Adulto Joven , China/epidemiología , Adolescente , Síndrome de Hipersensibilidad a Medicamentos/etiología
2.
Front Immunol ; 15: 1414136, 2024.
Artículo en Inglés | MEDLINE | ID: mdl-39072330

RESUMEN

Introduction: Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are rare yet life-threatening adverse events associated with immune checkpoint inhibitors (ICIs). This systematic review synthesizes the current literature to elucidate the clinical characteristics and outcomes of patients with ICI-related SJS/TEN. Methods: We conducted a thorough search across databases including Embase, Web of Science, Cochrane, MEDLINE, Scopus, and PubMed. Selection criteria focused on reports of SJS/TEN among cancer patients treated with ICIs, analyzing clinical manifestations, therapeutic interventions, and outcomes. Results: Our analysis included 47 articles involving 50 patients with ICI-related SJS/TEN. The cohort had a mean age of 63 years, with a slight male predominance (54%). Most patients had melanoma or non-small cell lung cancer. SJS/TEN typically occurred early, with a median onset of 23 days post-ICI initiation. Treatment primarily involved systemic corticosteroids and intravenous immunoglobulins. The overall mortality rate was 20%, higher for TEN at 32%, with infections and tumor progression as leading causes. Median time from onset to death was 28 days. Survivors experienced a median re-epithelization time of 30 days, positively correlated with the extent of epidermal detachment (rs = 0.639, p = 0.009). Deceased patients exhibited a significantly higher proportion of TEN (90% vs. 48%, p = 0.029) and a larger epidermal detachment area (90% vs. 30% of the body surface area [BSA], p = 0.005) compared to survivors. The combination therapy group showed a higher proportion of TEN compared to corticosteroid monotherapy or non-corticosteroid therapy groups (72% vs. 29% and 50%, p = 0.01), with no significant differences in mortality or re-epithelization time. Dual ICI therapy resulted in a higher TEN rate than single therapy (100% vs. 50%, p = 0.028). Among single ICI therapies, the sintilimab-treated group trended towards a higher TEN rate (75% vs. 40-50%, p = 0.417), a larger detachment area (90% vs. 30-48% of BSA, p = 0.172), and a longer re-epithelization time (44 vs. 14-28 days, p = 0.036) compared to other ICI groups, while mortality rates remained similar. Conclusion: ICI-related SJS/TEN substantially impacts patient outcomes. Prospective clinical trials are critically needed to further clarify the pathogenesis and optimize therapeutic regimens.


Asunto(s)
Inhibidores de Puntos de Control Inmunológico , Síndrome de Stevens-Johnson , Humanos , Síndrome de Stevens-Johnson/etiología , Síndrome de Stevens-Johnson/mortalidad , Inhibidores de Puntos de Control Inmunológico/efectos adversos , Masculino , Femenino , Persona de Mediana Edad , Neoplasias/tratamiento farmacológico , Neoplasias/mortalidad
3.
Hong Kong Med J ; 30(2): 102-109, 2024 Apr.
Artículo en Inglés | MEDLINE | ID: mdl-38531617

RESUMEN

INTRODUCTION: Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) [hereafter, SJS/TEN] are uncommon but severe mucocutaneous reactions. Although they have been described in many populations worldwide, data from Hong Kong are limited. Here, we explored the epidemiology, disease characteristics, aetiology, morbidity, and mortality of SJS/TEN in Hong Kong. METHODS: This retrospective cohort study included all hospitalised patients who had been diagnosed with SJS/TEN in Prince of Wales Hospital from 1 January 2004 to 31 December 2020. RESULTS: There were 125 cases of SJS/TEN during the 17-year study period. The annual incidence was 5.07 cases per million. The mean age at onset was 51.4 years. The mean maximal body surface area of epidermal detachment was 23%. Overall, patients in 32% of cases required burns unit or intensive care unit admission. Half of the cases involved concomitant sepsis, and 23.2% of cases resulted in multiorgan failure or disseminated intravascular coagulation. The mean length of stay was 23.9 days. The cause of SJS/TEN was attributed to a drug in 91.9% of cases, including 84.2% that involved anticonvulsants, allopurinol, antibiotics, or analgesics. In most cases, patients received treatment comprising either best supportive care alone (35.2%) or combined with intravenous immunoglobulin (43.2%). The in-hospital mortality rate was 21.6%. Major causes of death were multiorgan failure and/or fulminant sepsis (81.5%). CONCLUSION: This study showed that SJS/TEN are uncommon in Hong Kong but can cause substantial morbidity and mortality. Early recognition, prompt withdrawal of offending agents, and multidisciplinary supportive management are essential for improving clinical outcomes.


Asunto(s)
Síndrome de Stevens-Johnson , Humanos , Síndrome de Stevens-Johnson/epidemiología , Síndrome de Stevens-Johnson/terapia , Síndrome de Stevens-Johnson/mortalidad , Síndrome de Stevens-Johnson/etiología , Hong Kong/epidemiología , Persona de Mediana Edad , Estudios Retrospectivos , Masculino , Femenino , Adulto , Incidencia , Anciano , Tiempo de Internación/estadística & datos numéricos , Alopurinol/efectos adversos , Anticonvulsivantes/efectos adversos , Anticonvulsivantes/uso terapéutico , Sepsis/epidemiología , Insuficiencia Multiorgánica/epidemiología , Insuficiencia Multiorgánica/etiología , Insuficiencia Multiorgánica/mortalidad
4.
BMC Med Inform Decis Mak ; 22(1): 273, 2022 10 19.
Artículo en Inglés | MEDLINE | ID: mdl-36261833

RESUMEN

BACKGROUND: Toxic epidermal necrolysis (TEN) patients require multi-directional and multi-disciplinary treatment. In most cases, they are hospitalised at intensive care units and require multi-directional, burn-complication preventive care. Choosing the most appropriate treatment option might be troublesome even when predicting scores are used. SCORTEN is the most renowned prognostic score for TEN patients, however, there are some data indicating that the accuracy of this test may be limited. The credibility of not just the predicted mortality risk, but also componential laboratory results and clinical features subject to debate. The aim of this study was to evaluate the efficacy and credibility of SCORTEN in clinical practice, on proprietary material. METHODS: A retrospective analysis of 35 patients with diagnosed in histopathology TEN was performed. The inclusion criteria were as follows: day of submission before 5th day from the onset of the symptoms, full protocol of plasmaphereses and IVIGs according to our scheme. Our protocol includes cycle of plasmapheresis with frozen fresh plasma twice daily for the first 2 days following admission, and once daily for the subsequent 5 to 7 days. IVIGs were administered after the first two sessions of plasmapheresis, for 4 to 7 days. The dosage was calculated according to body weight, at 0.4 to 0.5 g/kg per dose. RESULTS: The sensitivity of SCORTEN for the analysed cohort was 100%, with a specificity of 24%. The estimated death was 41,9%, while the actual death rates were 12,5%. Our protocol improved the survival, OR = 26,57, RR = 6,34, p = 0,022. Decrease in mortality was caused by a combined treatment protocol we use- plasmaphereses with IVIGs. No independent risk factor was significant in death evaluation. CONCLUSION: Our data suggest that the scoring system for predicting death among TEN patients are reliable when they are high. New prognostic factors should be found to improve the evaluation of patients with low SCORTEN.


Asunto(s)
Síndrome de Stevens-Johnson , Humanos , Mortalidad Hospitalaria , Unidades de Cuidados Intensivos , Estudios Retrospectivos , Índice de Severidad de la Enfermedad , Síndrome de Stevens-Johnson/diagnóstico , Síndrome de Stevens-Johnson/mortalidad , Síndrome de Stevens-Johnson/terapia
5.
J Wound Care ; 30(6): 492-496, 2021 Jun 02.
Artículo en Inglés | MEDLINE | ID: mdl-34121430

RESUMEN

OBJECTIVE: Stevens-Johnson syndrome (SJS) and its more severe counterpart, toxic epidermal necrolysis (TEN), are skin hypersensitivity reactions defined by epidermal blistering and necrosis. The exact pathophysiology of SJS/TEN is yet to be deciphered, but a number of risk factors have been identified including adverse drug reactions. The diagnosis of SJS/TEN is made on a clinical basis, and treatment consists of supportive care and occasionally immunosuppressants, such as cyclosporin, high-dose intravenous immunoglobulins and/or corticosteroids. Mortality rates can reach 20-25% in adults but are reduced with early intervention. To identify optimal treatment regimens, to better understand the patient cohort affected, and to help identify key risk factors for mortality, we report our experience with the treatment and management of SJS/TEN patients. METHODS: A retrospective review of consecutive patients with SJS and/or TEN admitted to a single burns centre in Germany, between 2008 and 2018, was conducted. The primary outcomes of demographics, clinical course, treatment and patient-reported outcomes were recorded and compared with a control group of patients with burns without a diagnosis of SJS/TEN. RESULTS: A total of 23 patients with SJS/TEN met the inclusion criteria: 17 (74%) with TEN; four (17%) with SJS/TEN overlap; and two (9%) with SJS. Of the patients, 14 (61%) were female and nine (39%) were male. Patient age ranged from 32-78 years (mean: 52 years). A matched cohort of 23 patients with burns served as the control group. All patients received standard of care with a multidisciplinary team. Compared with the control group, SJS/TEN patients had higher mortality rates (n=6, 26% versus n=8, 35%, respectively). The average age of death was 69 years in SJS/TEN patients versus 63 years in control group patients. Age and SCORTEN scores were significant predictors of mortality. CONCLUSIONS: SJS and TEN are rare but extreme reactions of the skin and mucosa, associated with high disease mortality rates. This 10-year single-centre retrospective review contributes to the bank of information for reviews evaluating the management of SJS/TEN patients.


Asunto(s)
Corticoesteroides/uso terapéutico , Quemaduras/terapia , Inmunoglobulinas Intravenosas/uso terapéutico , Inmunosupresores/uso terapéutico , Síndrome de Stevens-Johnson/tratamiento farmacológico , Cicatrización de Heridas , Adulto , Anciano , Unidades de Quemados , Quemaduras/mortalidad , Femenino , Alemania/epidemiología , Humanos , Masculino , Persona de Mediana Edad , Estudios Retrospectivos , Síndrome de Stevens-Johnson/mortalidad , Resultado del Tratamiento
6.
Med Clin North Am ; 105(4): 577-597, 2021 Jul.
Artículo en Inglés | MEDLINE | ID: mdl-34059239

RESUMEN

Severe cutaneous adverse reactions to medications (SCARs) include drug reaction with eosinophilia and systemic symptoms, Stevens-Johnson syndrome, toxic epidermal necrolysis, and acute generalized exanthematous pustulosis. They are all non-immunoglobulin E mediated hypersensitivity reaction patterns, distinguished from simple cutaneous drug eruptions by immunologic pathogenesis and internal organ involvement. Herein the clinical features, diagnostic workup, and management considerations are presented for each of these major SCARs.


Asunto(s)
Pustulosis Exantematosa Generalizada Aguda/patología , Efectos Colaterales y Reacciones Adversas Relacionados con Medicamentos/patología , Eosinofilia/diagnóstico , Síndrome de Stevens-Johnson/etiología , Síndrome de Stevens-Johnson/patología , Pustulosis Exantematosa Generalizada Aguda/diagnóstico , Pustulosis Exantematosa Generalizada Aguda/etiología , Corticoesteroides/administración & dosificación , Corticoesteroides/uso terapéutico , Adulto , Anciano , Diagnóstico Diferencial , Efectos Colaterales y Reacciones Adversas Relacionados con Medicamentos/diagnóstico , Efectos Colaterales y Reacciones Adversas Relacionados con Medicamentos/epidemiología , Efectos Colaterales y Reacciones Adversas Relacionados con Medicamentos/terapia , Eosinofilia/inducido químicamente , Femenino , Humanos , Hipersensibilidad/inmunología , Masculino , Persona de Mediana Edad , Farmacogenética , Pronóstico , Factores de Riesgo , Índice de Severidad de la Enfermedad , Síndrome de Stevens-Johnson/diagnóstico , Síndrome de Stevens-Johnson/mortalidad
7.
J Am Acad Dermatol ; 85(4): 873-877, 2021 10.
Artículo en Inglés | MEDLINE | ID: mdl-33940101

RESUMEN

BACKGROUND: Age, bicarbonate, cancer, dialysis, 10% body surface area risk model (ABCD-10) has recently been proposed as an alternative to the SCORe of toxic epidermal necrolysis (SCORTEN) model for predicting in-hospital mortality in patients with Stevens-Johnson syndrome and toxic epidermal necrolysis (SJS/TEN). In contrast to SCORTEN, ABCD-10 incorporates prior dialysis and upweights the impact of cancer. OBJECTIVE: To determine the performance of ABCD-10 compared with that of SCORTEN in mortality prediction at a large, tertiary burn center. METHODS: A retrospective analysis of 192 patients with SJS/TEN admitted to the North Carolina Jaycee Burn Center from January 1, 2009, to December 31, 2019, was conducted. Data on these patients were collected using the burn registry and a manual chart review. The performance of both the mortality prediction models was assessed using univariate logistic regression and the Hosmer-Lemeshow test. RESULTS: The overall mortality was 22% (n = 43). Nine (5%) patients had cancer, and 7 (4%) had undergone prior dialysis; neither factor was associated with mortality (P = .11 and P = .62, respectively). SCORTEN was well calibrated to predict inpatient mortality (P = .82), whereas ABCD-10 appeared to have a poorer fit (P < .001) in these patients. Both the models showed good discrimination. LIMITATIONS: Small sample size. CONCLUSION: SCORTEN was a better predictor of inpatient mortality than ABCD-10 in a North American cohort of patients treated at the tertiary burn center.


Asunto(s)
Síndrome de Stevens-Johnson , Unidades de Quemados , Estudios de Cohortes , Humanos , Estudios Retrospectivos , Índice de Severidad de la Enfermedad , Síndrome de Stevens-Johnson/mortalidad
8.
J Invest Dermatol ; 141(5): 1139-1140, 2021 05.
Artículo en Inglés | MEDLINE | ID: mdl-33888216

RESUMEN

In-hospital mortality for epidermal necrolysis (EN) has been well-characterized, but less is known about the long-term complications. Marxer et al. (2020) report mortality rates of 7.4% during the initial hospitalization, 4.8% within 90 days, and 7.6% after 91 days. Compared with that of matched controls, long-term mortality was not increased, highlighting the importance of understanding the long-term sequelae of EN survivors.


Asunto(s)
Síndrome de Stevens-Johnson/complicaciones , Mortalidad Hospitalaria , Hospitalización , Humanos , Calidad de Vida , Síndrome de Stevens-Johnson/mortalidad , Síndrome de Stevens-Johnson/psicología
9.
Indian J Dermatol Venereol Leprol ; 87(5): 628-644, 2021.
Artículo en Inglés | MEDLINE | ID: mdl-33871208

RESUMEN

BACKGROUND: Limited evidence is available about effectiveness and choice of immunomodulating treatment modalities for toxic epidermal necrolysis (TEN). AIMS: To compare the effectiveness of interventions to reduce mortality in patients of toxic epidermal necrolysis through network meta-analysis. METHODS: Studies were retrieved using PubMed, Google Scholar and Cochrane Database of Systematic Reviews from inception to September 18, 2018. Only English language articles were considered. Observational and randomized controlled studies having ≥ 5 TEN patients in each intervention arm were included. Two investigators independently extracted study characteristics, intervention details and mortality data. Bayesian network meta-analysis was performed using the Markov chain Monte Carlo (MCMC) approach through the random effect model. The ranking analysis was done to provide a hierarchy of interventions. The consistency between direct and indirect evidence was assessed through node spit analysis. The primary outcome was to compare the mortality [Odds ratio OR (95% credibility interval CrI)] among all treatment modalities of TEN. RESULTS: Twenty-four studies satisfying the selection criteria were included. The network analysis showed improved survival with cyclosporine as compared to supportive care [OR- 0.19 (95% CrI: 0.05, 0.59)] and intravenous immunoglobulin [OR- 0.21 (95% CrI: 0.05, 0.76)]. The hierarchy of treatments based on "surface under the cumulative ranking curves" (SUCRA) value were cyclosporine (0.93), steroid+intravenous immunoglobulin (0.76), etanercept (0.59), steroids (0.46), intravenous immunoglobulin (0.40), supportive care (0.34) and thalidomide (0.02). No inconsistencies between direct and indirect estimates were observed for any of the treatment pairs. LIMITATIONS: Evidence is mainly based on retrospective studies. CONCLUSION: The use of cyclosporine can reduce mortality in TEN patients. Other promising immunomodulators could be steroid+intravenous immunoglobulin combination and etanercept.


Asunto(s)
Síndrome de Stevens-Johnson/terapia , Ciclosporina/uso terapéutico , Fármacos Dermatológicos/uso terapéutico , Etanercept/uso terapéutico , Glucocorticoides/uso terapéutico , Humanos , Inmunoglobulinas Intravenosas/uso terapéutico , Factores Inmunológicos/uso terapéutico , Inmunosupresores/uso terapéutico , Síndrome de Stevens-Johnson/mortalidad
12.
J Am Acad Dermatol ; 84(6): 1782-1791, 2021 Jun.
Artículo en Inglés | MEDLINE | ID: mdl-32828861

RESUMEN

BACKGROUND: Patients with Stevens-Johnson syndrome (SJS)/toxic epidermal necrolysis (TEN) have high mortality rates. Disseminated intravascular coagulation has been reported in SJS/TEN patients. The influence of this lethal complication in patients with SJS/TEN is not well known. OBJECTIVE: This study aimed to investigate the risk and outcomes of disseminated intravascular coagulation in patients with SJS/TEN. METHODS: We analyzed the disseminated intravascular coagulation profiles of patients receiving a diagnosis of SJS/TEN between 2010 and 2019. RESULTS: We analyzed 150 patients with SJS/TEN (75 with SJS, 22 with overlapping SJS/TEN, and 53 with TEN) and their complete disseminated intravascular coagulation profiles. Disseminated intravascular coagulation was diagnosed in 32 patients (21.3%), primarily those with TEN. It was significantly associated with systemic complications, including gastrointestinal bleeding, respiratory failure, renal failure, liver failure, infection, and bacteremia. Additionally, SJS/TEN patients with disseminated intravascular coagulation had elevated procalcitonin levels. Among patients with SJS/TEN, disseminated intravascular coagulation was associated with a greater than 10-fold increase in mortality (78.1% vs 7%). LIMITATIONS: The study limitations include small sample size and a single hospital system. CONCLUSION: Disseminated intravascular coagulation is a potential complication of SJS/TEN and associated with higher mortality. Early recognition and appropriate management of this critical complication are important for patients with SJS/TEN.


Asunto(s)
Coagulación Intravascular Diseminada/etiología , Coagulación Intravascular Diseminada/mortalidad , Hemorragia Gastrointestinal/complicaciones , Síndrome de Stevens-Johnson/complicaciones , Síndrome de Stevens-Johnson/mortalidad , Adulto , Anciano , Anciano de 80 o más Años , Bacteriemia/complicaciones , Bacteriemia/microbiología , Femenino , Humanos , Estimación de Kaplan-Meier , Fallo Hepático/complicaciones , Masculino , Persona de Mediana Edad , Insuficiencia Renal/complicaciones , Insuficiencia Respiratoria/complicaciones , Tasa de Supervivencia
13.
Int J Dermatol ; 60(1): 44-52, 2021 Jan.
Artículo en Inglés | MEDLINE | ID: mdl-32686136

RESUMEN

BACKGROUND: Toxic epidermal necrolysis (TEN) is a life-threatening severe cutaneous adverse reaction. Data on pediatric TEN is limited. METHODS: Case records of 44 children, 1 month-12 years with a diagnosis of TEN (>30% body surface area [%BSA] detachment) admitted to a tertiary pediatric intensive care unit (PICU) between 2009 and 2018 were analyzed retrospectively. The primary outcome was mortality, and secondary outcomes were organ dysfunction, length of stay (LOS), and long-term sequelae. RESULTS: Median (IQR) age was 6.5 (3.6, 8.0) years, and 25 (57%) were boys. Median (IQR) %BSA involved, SCORTEN score, and PRISM-III were 65% (45, 80); 2 (2, 3) and 13 (10, 16), respectively. Antiepileptics (n = 24, 54.6%) and antimicrobials (n = 8, 18.2%) were the most common offending agents. Twenty-four (54.5%) children had culture positive sepsis. Immunomodulatory therapy was provided in 35 (79.5%) and conservative management in nine (20.5%) children. Intravenous immunoglobulin (IVIG) was given in 22 (50%), steroids in three (6.8%), and both IVIG and steroids in 10 (22.7%) children. Respiratory failure (n = 14, 31.8%) was the commonest organ failure. Mortality was 15.9% (n = 7), and median (IQR) PICU-LOS in survivors was 8 (4, 11.75) days. There was no association between IVIG, steroids, or conservative management with mortality or LOS. Ocular sequelae (n = 20, 54.1%) were the most common long-term complication followed by skin (18, 40.1%). CONCLUSION: Immunomodulation with IVIG or steroids was not associated with any mortality benefit as compared to conservative management alone. Further research is required to determine the most effective treatment in pediatric TEN.


Asunto(s)
Cuidados Críticos , Síndrome de Stevens-Johnson/terapia , Antiinflamatorios/uso terapéutico , Niño , Preescolar , Tratamiento Conservador , Dexametasona/uso terapéutico , Oftalmopatías/etiología , Femenino , Humanos , Inmunoglobulinas Intravenosas/uso terapéutico , Factores Inmunológicos/uso terapéutico , Inmunomodulación , Lactante , Unidades de Cuidado Intensivo Pediátrico , Tiempo de Internación , Masculino , Metilprednisolona/uso terapéutico , Insuficiencia Respiratoria/etiología , Estudios Retrospectivos , Sepsis/microbiología , Índice de Severidad de la Enfermedad , Esteroides/uso terapéutico , Síndrome de Stevens-Johnson/complicaciones , Síndrome de Stevens-Johnson/mortalidad , Tasa de Supervivencia , Factores de Tiempo , Resultado del Tratamiento
14.
J Am Acad Dermatol ; 84(3): 644-653, 2021 Mar.
Artículo en Inglés | MEDLINE | ID: mdl-32561372

RESUMEN

BACKGROUND: Complications involving internal organs are usually present in Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN). However, pancreatic complications are rarely reported and studied. OBJECTIVE: To summarize clinical characteristics of SJS/TEN-associated acute pancreatic injuries and to investigate underlying inflammatory mechanisms. METHODS: Clinical records of 124 inpatients with SJS/TEN were reviewed. Serum levels of tumor necrosis factor α, interleukin (IL) 6, IL-18, IL-15, IL-12p70, and soluble CD56 were determined in 18 healthy donors and 17 patients with SJS/TEN, including 3 with acute pancreatic injuries. RESULTS: Acute pancreatic injury was diagnosed in 7.3% of patients (9/124) in the SJS/TEN cohort. Elevation of serum transaminase level and hypoalbuminemia occurred more frequently in patients with acute pancreatic injuries compared with those without pancreatic symptoms (P = .004 and <.001, respectively). Although acute pancreatic injury did not alter mortality rate of SJS/TEN, it was associated with longer hospitalization stays (P = .008). Within the serum cytokines whose levels were elevated in SJS/TEN, only IL-18 was found to be selectively increased in patients with acute pancreatic injuries compared with those without them (P = .03). LIMITATIONS: Cohort was small. CONCLUSION: Acute pancreatic injury is a gastrointestinal complication of SJS/TEN in which hepatotoxicity is more likely to occur. Overexpression of IL-18 might be involved in this unique entity.


Asunto(s)
Interleucina-18/sangre , Pancreatitis/inmunología , Síndrome de Stevens-Johnson/complicaciones , Adolescente , Adulto , Anciano , Antígeno CD56/sangre , Antígeno CD56/inmunología , Niño , Femenino , Humanos , Interleucina-12/sangre , Interleucina-12/inmunología , Interleucina-15/sangre , Interleucina-15/inmunología , Interleucina-18/inmunología , Interleucina-6/sangre , Interleucina-6/inmunología , Masculino , Persona de Mediana Edad , Pancreatitis/sangre , Estudios Retrospectivos , Síndrome de Stevens-Johnson/sangre , Síndrome de Stevens-Johnson/inmunología , Síndrome de Stevens-Johnson/mortalidad , Factor de Necrosis Tumoral alfa/sangre , Factor de Necrosis Tumoral alfa/inmunología , Adulto Joven
15.
J Am Acad Dermatol ; 84(2): 390-397, 2021 Feb.
Artículo en Inglés | MEDLINE | ID: mdl-32898587

RESUMEN

BACKGROUND: Various systemic immunomodulating therapies have been used to treat toxic epidermal necrolysis (TEN), but their efficacy remains unclear. OBJECTIVE: To perform a systematic review and network meta-analysis (NMA) evaluating the effects of systemic immunomodulating therapies on mortality for Stevens-Johnson syndrome (SJS)/TEN overlap and TEN. METHODS: A literature search was performed in online databases (from inception to October 31, 2019). Outcomes were mortality rates and Score of Toxic Epidermal Necrolysis (SCORTEN)-based standardized mortality ratio (SMR). A frequentist random-effects model was adopted. RESULTS: Sixty-seven studies involving 2079 patients were included. An NMA of 10 treatments showed that none was superior to supportive care in reducing mortality rates and that thalidomide was associated with a significantly higher mortality rate (odds ratio, 11.67; 95% confidence interval [CI], 1.42-95.96). For SMR, an NMA of 11 treatment arms showed that corticosteroids and intravenous immunoglobulin combination therapy was the only treatment with significant survival benefits (SMR, 0.53; 95% CI, 0.31-0.93). LIMITATIONS: Heterogeneity and a paucity of eligible randomized controlled trials. CONCLUSIONS: Combination therapy with corticosteroids and IVIg may reduce mortality risks in patients with SJS/TEN overlap and TEN. Cyclosporine and etanercept are promising therapies, but more studies are required to provide clearer evidence.


Asunto(s)
Glucocorticoides/uso terapéutico , Inmunoglobulinas Intravenosas/uso terapéutico , Factores Inmunológicos/uso terapéutico , Síndrome de Stevens-Johnson/terapia , Quimioterapia Combinada/métodos , Humanos , Metaanálisis en Red , Ensayos Clínicos Controlados Aleatorios como Asunto , Síndrome de Stevens-Johnson/mortalidad , Talidomida/uso terapéutico , Resultado del Tratamiento
17.
Allergol Int ; 70(2): 229-234, 2021 Apr.
Artículo en Inglés | MEDLINE | ID: mdl-33279401

RESUMEN

BACKGROUND: Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are rare but life-threatening disorders characterized by widespread epidermal necrosis of the skin and mucosa. The severity-of-illness scoring system for TEN (SCORTEN) was widely used since 2000 as a standard prognostic tool consisting of seven clinical values. METHODS: To evaluate the prognosis using current treatments and risk factors for mortality, we retrospectively analyzed 59 cases of TEN, including SJS/TEN overlap treated in two university hospitals from January 2000 to March 2020. RESULTS: The mortality rate of TEN was 13.6% (8/59). All patients treated with high-dose steroid administration in combination with plasma exchange and/or immunoglobulin therapy recovered. Logistic regression analysis showed nine clinical composite scores, namely: heart rate (≧120 bpm), malignancy present, percentage of body surface area with epidermal detachment (>10%), blood urea nitrogen (>28 mg/dL), serum bicarbonate level (<20 mEq/L), serum glucose level (>252 mg/dL), age (≧71 years), the interval between disease onset and treatment initiation at the specialty hospital (≧8 days), and respiratory disorder within 48 h after admission. The receiver operating characteristic curves confirmed a high potential for predicting the prognosis of TEN. CONCLUSIONS: Recent developments in treatment strategies have contributed to the improved prognosis of TEN patients. A modified severity scoring model composed of nine scores may be helpful in the prediction of TEN prognosis in recent patients. Further large-scale studies are needed to confirm mortality findings to improve prognostication in patients with TEN.


Asunto(s)
Síndrome de Stevens-Johnson/mortalidad , Adolescente , Corticoesteroides/uso terapéutico , Adulto , Anciano , Anciano de 80 o más Años , Antibacterianos/efectos adversos , Antiinflamatorios no Esteroideos/efectos adversos , Anticonvulsivantes/efectos adversos , Niño , Preescolar , Estudios de Cohortes , Femenino , Humanos , Japón/epidemiología , Masculino , Persona de Mediana Edad , Pronóstico , Factores de Riesgo , Índice de Severidad de la Enfermedad , Síndrome de Stevens-Johnson/tratamiento farmacológico , Adulto Joven
19.
Int J Dermatol ; 60(1): 53-59, 2021 Jan.
Artículo en Inglés | MEDLINE | ID: mdl-33252832

RESUMEN

BACKGROUND: To retrospectively review the outcomes of two rare cutaneous diseases, Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN), and to question the practice of averaging the mortality rate on the assumption that they are one disease. METHODS: A retrospective chart review of all patients diagnosed with SJS and TEN by a dermatologist between January 1, 2000, and January 1, 2020, at our institution was performed. Seventy-one patients were identified (21 pediatric and 50 adults). Pathology slides from 32 adult patients (64%) were evaluated by a blinded board-certified dermatopathologist. RESULTS: Of the adult patients, 31 had SJS, two had SJS-TEN overlap, and 17 had TEN. All 21 patients in the pediatric group were diagnosed with SJS mainly caused by Mycoplasma. Mortality rates were 6.5% for SJS among adults and 35.3% for TEN. Chemotherapy-induced TEN is a trigger with 50% mortality. CONCLUSIONS: SJS was more common in adults and pediatric cases than TEN (3:1) and had a much better prognosis and outcome. Combining and averaging the mortality rates of TEN and SJS are not advised as SJS is mainly a mucocutaneous disorder with good prognosis versus TEN, a systemic toxicity of multiple organs with deep skin detachment.


Asunto(s)
Síndrome de Stevens-Johnson/etiología , Síndrome de Stevens-Johnson/mortalidad , Adolescente , Adulto , Factores de Edad , Antibacterianos/efectos adversos , Antiinflamatorios/uso terapéutico , Anticonvulsivantes/efectos adversos , Niño , Preescolar , Femenino , Humanos , Inmunoglobulinas Intravenosas/uso terapéutico , Factores Inmunológicos/uso terapéutico , Masculino , Persona de Mediana Edad , Infecciones por Mycoplasma/complicaciones , Pronóstico , Estudios Retrospectivos , Índice de Severidad de la Enfermedad , Esteroides/uso terapéutico , Síndrome de Stevens-Johnson/patología , Síndrome de Stevens-Johnson/terapia , Adulto Joven
20.
Rev. cuba. med ; 59(4): e53, oct.-dic. 2020. graf
Artículo en Español | LILACS, CUMED | ID: biblio-1144510

RESUMEN

Se estudiaron dos pacientes ingresados en sala de cuidados intensivos polivalente, los cuales presentaron necrolisis tóxica epidémica (NTE), complicación poco frecuente que tiene una incidencia de 1 a 4 casos por millón de habitantes. La causa de la enfermedad en estos pacientes fue medicamentosa y los principales medicamentos implicados fueron anfotericin B y ciprofloxacino. Una de las cuestiones que hace interesante el reporte de estas reacciones adversas es que se trata del primer caso de NTE relacionada con el uso de Anfotericin B Liposomal que se reporta en Cuba y en el resto de la comunidad científica. El diagnóstico se confirmó por biopsia de piel, ambos pacientes presentaron un riesgo de muerte, según índice de Scorten, mayor al 50 por ciento. Por lo infrecuente que es el tratamiento de esta afección en una sala de cuidados intensivos polivalentes, por lo raro que es ver esta enfermedad en relación a estos antibióticos antes mencionados, por lo dramático del cuadro clínico y por la evolución tórpida de los pacientes, se realizó esta investigación con el objetivo de transmitir la experiencia y el conocimiento a la comunidad médica y científica en general y a los profesionales que laboran en las salas de terapia intensiva polivalente en particular(AU)


Two patients admitted to a multipurpose intensive care ward were studied. They showed epidemic toxic necrolysis (NTE), a rare complication that has an incidence of 1 to 4 cases per million inhabitants. Drugs were the cause of the disease in these patients and the main drugs involved were amphotericin B and ciprofloxacin. This is the first case of NTE related to the use of Liposomal Amphotericin B reported in Cuba and in the rest of the scientific community, which makes it interesting. The diagnosis was confirmed by skin biopsy, both patients had risk of death, according to the Scorten index, higher than 50 percent. This research was carried out with the aim of communicating the experience and knowledge to the medical and scientific community in general and to the professionals who work in multipurpose intensive care rooms in particular, since this condition is rare in a multipurpose intensive care room, it is rare in relation to the aforementioned antibiotics, its dramatic clinical status and the torpid evolution of patients(AU)


Asunto(s)
Humanos , Femenino , Persona de Mediana Edad , Síndrome de Stevens-Johnson/mortalidad , Efectos Colaterales y Reacciones Adversas Relacionados con Medicamentos/mortalidad , Unidades de Cuidados Intensivos , Cuba
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