Materiały źródłowe

• #1 Epidemiology of Stevens-Johnson syndrome and toxic epidermal necrolysis in the United States and factors predictive of outcome

  https://pmc.ncbi.nlm.nih.gov/articles/PMC10413346/

  Stevens-Johnson syndrome (SJS), toxic epidermal necrolysis (TEN), and SJS-TEN overlap syndrome are rare severe cutaneous adverse reactions associated with high mortality. […] To estimate incidence and describe trends of SJS/TEN hospitalizations in the United States and to describe the clinical, demographic, and geographic characteristics of affected patients and risk factors for mortality. […] We identified 51,040 hospitalizations involving SJS/TEN. Among those, 37,283 (73.0%) were for SJS only, 7818 (15.3%) were for SJS-TEN overlap syndrome, and 7160 (14.0%) were for TEN only. Overall, SJS/TEN hospitalization rates declined over time, 2010 to 2020 (P .05). Mortality rates of the SJS group, SJS-TEN overlap syndrome group, and TEN group were 5.4%, 14.4%, and 15.3%, respectively. […] Risk factors identified in this study lay the groundwork for improvement in SJS/TEN mortality prediction scoring.

• #2 Stevens–Johnson Syndrome and Toxic Epidermal Necrolysis: A Review of Diagnosis and Management

  https://www.mdpi.com/1648-9144/57/9/895

  Stevens–Johnson Syndrome (SJS) and Toxic Epidermal Necrolysis (TEN) are rare diseases that are characterized by widespread epidermal necrosis and sloughing of skin. They are associated with significant morbidity and mortality, and early diagnosis and treatment is critical in achieving favorable outcomes for patients. […] These are rare diseases and reported incidence rates vary by location. Frey et al. reported an incidence of 5.76 cases of SJS/TEN per million persons per year in the UK from 1995–2013. Hsu et al. reported 9.2, 1.6, and 1.9 cases per million adults per year in the US from 2009–2012 for SJS, SJS/TEN, and TEN, respectively. Yang et al. reported incidence rates in Korea from 2009–2013 as 3.96–5.03 and 0.94–1.45 per million persons per year for SJS and TEN, respectively. […] The mortality rates are 4.8–9% for SJS, 19.4–29% for SJS/TEN, and 14.8–48% for TEN.

• #3 Stevens-Johnson Syndrome (SJS) and Toxic Epidermal Necrolysis (TEN) – Dermatologic Disorders – Merck Manual Professional Edition

  https://www.merckmanuals.com/professional/dermatologic-disorders/hypersensitivity-and-reactive-skin-disorders/stevens-johnson-syndrome-sjs-and-toxic-epidermal-necrolysis-ten

  Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are clinically similar except for their distribution. By one commonly accepted definition, changes affect 10% of body surface area in SJS and 30% of body surface area in TEN; involvement of 10 to 30% of body surface area is considered SJS/TEN overlap. […] The disorders affect between 2 and 9 people/million. Incidence, severity, or both of these disorders may be higher in recipients of bone marrow transplants, in patients with HIV who have Pneumocystis jirovecii infection, in patients with systemic lupus erythematosus, and in patients with other chronic rheumatologic diseases. […] Mortality can be as high as 25 to 35% in adults but tends to be lower in children and with early treatment. With early therapy, survival rates for TEN approach 90%. The severity-of-illness score for toxic epidermal necrolysis (SCORTEN) systematically scores 7 independent risk factors within the first 24 hours of presentation to the hospital to determine the mortality rate for a particular patient.

• #4 Stevens-Johnson Syndrome – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK459323/

  Stevens-Johnson syndrome/toxic epidermal necrolysis is estimated to affect two to seven per million people each year. Stevens-Johnson syndrome is three times more common than toxic epidermal necrolysis. Stevens-Johnson syndrome/toxic epidermal necrolysis can affect anyone with a genetic predisposition: any age, either sex, and all races, although it is more common in older people and women. It is much more likely to occur in people infected with the human immunodeficiency virus (HIV), with an estimated incidence of 1/1000. […] Numerous medications have been reported to trigger Stevens-Johnson syndrome/toxic epidermal necrolysis. […] Genetic factors include human leukocyte antigen (HLA) allotypes that lead to an increased risk of Stevens-Johnson syndrome/toxic epidermal necrolysis when exposed to aromatic anticonvulsants and allopurinol. Family members of a patient with Stevens-Johnson syndrome/toxic epidermal necrolysis should be advised that they are at risk of developing the disease and should be cautious about taking any medications associated with the disease.

• #5 Stevens-Johnson syndrome/toxic epidermal necrolysis: MedlinePlus GeneticsLock

  https://medlineplus.gov/genetics/condition/stevens-johnson-syndrome-toxic-epidermal-necrolysis/

  SJS/TEN is a rare disease, affecting 1 to 2 per million people each year. Stevens-Johnson syndrome (the less severe form of the condition) is more common than toxic epidermal necrolysis. […] People who are HIV-positive and those with a chronic inflammatory disease called systemic lupus erythematosus are more likely to develop SJS/TEN than the general population. The reason for the increased risk is unclear, but immune system factors and exposure to multiple medications may play a role.

• #6 Stevens–Johnson syndrome – Wikipedia

  https://en.wikipedia.org/wiki/Stevens%E2%80%93Johnson_syndrome

  SJS is a rare condition, with a reported incidence of around 2.6 to 6.1 cases per million people per year. […] In the United States, about 300 new diagnoses are made each year. The condition is more common in adults than in children.

• #7 Stevens-Johnson Syndrome: Practice Essentials, Background, Pathophysiology

  https://emedicine.medscape.com/article/1197450-overview

  Strom et al reviewed Medicaid billing data from 1980-1984 in Michigan, Minnesota, and Florida to determine the incidence of Stevens-Johnson syndrome; the incidence rates were 7.1, 2.6, and 6.8 cases per million population per year, respectively. […] Cases tend to have a propensity for the early spring and winter. […] For overlapping SJS and TEN, oxicam NSAIDs (piroxicam, meloxicam, tenoxicam) and sulfonamides are most commonly implicated in the United States and other western nations. […] SJS occurs with a worldwide distribution similar in etiology and occurrence to that in the United States. However, a study from Germany reported only 1.1 cases per 1 million person-years. […] In contrast to the drugs most often implicated in western nations, allopurinol is the most common offending agent in Southeast Asian nations, including Malaysia, Singapore, Taiwan, and Hong Kong.

• #8 Azthena logo with the word Azthena

  https://www.news-medical.net/health/What-is-Stevens-Johnson-Syndrome.aspx

  Stevens-Johnson syndrome is predicted to impact two to seven million people each year. […] Stevens-Johnson syndrome occurs three times as frequently as toxic epidermal necrolysis. […] SJS is more common in elderly persons and women. It has an estimated prevalence of 1/1000 and is significantly more likely to occur among people infected with the human immunodeficiency virus (HIV). […] SJS mortality rates have been reported to vary from 25% to 70%, with significant morbidity.

• #9 Life-threatening dermatoses: Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis. Impact on the Spanish public health system (2010-2015) | PLOS One

  https://journals.plos.org/plosone/article?id=10.1371/journal.pone.0198582

  Stevens-Johnson Syndrome (SJS) and Toxic Epidermal Necrolysis (TEN) are serious mucocutaneous reactions. In Spain, the epidemiology and resulting expenses of these diseases are not well established. […] A total of 1,468 patients were recorded, 773 were men (52.7%). The mean age ( SD) was 52.25 26.15 years. The mean incidence rate for all diagnoses was 5.19 cases per million person-years (2.96 in SJS, 0.31 in SJS/TEN and 1.90 in TEN). […] The overall mortality of both diseases is approximately 10%, and clinical diagnosis and age were the variables with the greatest influence on mortality. This study describes a stable incidence and a similar prevalence to other European countries. Additionally, the data show a high cost due to hospitalizations. Finally, the CMBD could be a good system of epidemiological analysis for the study of infrequent diseases and hospital management of conditions such as SJS and TEN.

• #10 Life-threatening dermatoses: Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis. Impact on the Spanish public health system (2010-2015) | PLOS One

  https://journals.plos.org/plosone/article?id=10.1371/journal.pone.0198582

  The aim of this study is to evaluate the impact of SJS and TEN in the Spanish National Health System during the period from 2010-2015 using CMBD data. Global incidence in Spain in our work was 6.24 x 106 cases per million person-years: 3.65 x106 cases per million person-years for SJS and 2.22 x 106 cases per million person-years for TEN.

• #11 Life-threatening dermatoses: Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis. Impact on the Spanish public health system (2010-2015) | PLOS One

  https://journals.plos.org/plosone/article?id=10.1371/journal.pone.0198582

  Epidemiological information on this group of diseases is very limited. The incidence of TEN and SJS described in some European countries such as France and Germany varies between 11.4 and 13 cases x 106 inhabitants/year, respectively, while the incidence is somewhat lower in Asian countries. […] The objective of this study is to evaluate the impact of SJS and TEN in the Spanish National Health System during the period from 2010-2015. […] The incidence rate in Spain obtained in this study for these three diagnoses together is 5.19 cases per million py (95% CI, 4.925.45): 2.96 cases/million py (95% CI, 2.763.16) in SJS, 1.90 cases/million py (95% CI, 1.742.07) in TEN, and 0.31 cases/million py (95% CI, 0.240.38) in SJS/TEN. […] The total cost of hospital care for patients is estimated at 11.576.456,18 and the average cost per patient (CI 95%) of 7.885,86 (7.287,56-8.484,16). The costs for diagnosis and the estimated cost per year included in the cohort are detailed in Table 5. There are significant differences (p0.001) between SJS, SJS/TEN, and TEN.

• #12 Stevens–Johnson syndrome and toxic epidermal necrolysis in Hong Kong | HKMJ

  https://www.hkmj.org/abstracts/v30n2/102.htm

  Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are uncommon but severe mucocutaneous reactions. […] Here, we explored the epidemiology, disease characteristics, aetiology, morbidity, and mortality of SJS/TEN in Hong Kong. […] The annual incidence was 5.07 cases per million. […] This study showed that SJS/TEN are uncommon in Hong Kong but can cause substantial morbidity and mortality. […] Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are rare severe cutaneous adverse reactions in Hong Kong, with a combined annual incidence of 5.07 cases per million. […] Stevens-Johnson syndrome and TEN cause considerable burdens on the Hong Kong healthcare system due to their prolonged length of stay, high demand for intensive care, and substantial mortality.

• #13 DOAJ Logotype

  https://doaj.org/article/566afd3bfab045abbb785e79a9facc3d

  Stevens-Johnson syndrome (SJS), toxic epidermal necrolysis (TEN), and SJS-TEN overlap syndrome are rare severe cutaneous adverse reactions associated with high mortality. […] To estimate incidence and describe trends of SJS/TEN hospitalizations in the United States and to describe the clinical, demographic, and geographic characteristics of affected patients and risk factors for mortality. […] We identified 51,040 hospitalizations involving SJS/TEN. Among those, 37,283 (73.0%) were for SJS only, 7818 (15.3%) were for SJS-TEN overlap syndrome, and 7160 (14.0%) were for TEN only. Overall, SJS/TEN hospitalization rates declined over time, 2010 to 2020 (P .05). Mortality rates of the SJS group, SJS-TEN overlap syndrome group, and TEN group were 5.4%, 14.4%, and 15.3%, respectively. Increasing age, chronic kidney disease, pneumonia, sepsis, and malignant neoplasm were all significantly associated with increased odds of mortality (P .05). Non-Hispanic White racial/ethnic identification was associated with decreased odds of mortality (P .05). […] Risk factors identified in this study lay the groundwork for improvement in SJS/TEN mortality prediction scoring.

• #14 Orphanet: Stevens-Johnson syndrome/toxic epidermal necrolysis spectrum

  https://www.orpha.net/en/disease/detail/95455

  On average, the annual incidence is 1/319,000 in Europe; however, it varies worldwide with a higher incidence observed in Asian populations and in the US. Females are more often affected than males. […] In 85% of cases, the disorder is triggered by a clearly identifiable drug allergy. […] Medication history will find a suspect drug in 85% of cases. […] HLA predisposition should be screened before prescription of carbamazepine (and other aromatic anti-epileptics) and allopurinol in Chinese Hans population; and before prescription of abacavir in all populations. […] Patients should be admitted (according to the severity) to a dermatology or an intensive care or burns unit as soon as the diagnosis is suspected. […] Reepithelialization is usually rapid (2-3 weeks). However, the prognosis for patients with extensive forms is poor (20-25% mortality).

• #15

  https://link.springer.com/article/10.1007/s40257-024-00889-6

  Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) comprise a spectrum of severe cutaneous adverse reactions (SCAR) with life-threatening acute effects and serious long-term sequelae. SJS/TEN has a reported incidence of 15 cases per 1,000,000 individuals annually and has a higher incidence in adults than pediatric patients, likely due to increased exposure to potential triggers. […] Incidence of SJS/TEN also varies by country, partly due to (1) differences in genetic background and (2) differing prescribing patterns. […] Reported mortality rates are as high as 34-50%, with mortality correlating with BSA involvement. […] This review aims to provide an overview and update on the pathogenesis, precipitating factors, presentation, diagnosis, and management of SJS/TEN.

• #16 Stevens-Johnson Syndrome – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK459323/

  To date, findings have included the risk of Stevens-Johnson syndrome/toxic epidermal necrolysis in: Han Chinese, Thai, Malaysian, and South Indian people if they carry HLA-B*1502 and take aromatic anticonvulsants. Han Chinese if they carry HLA-B*5801 and take allopurinol Europeans if they carry HLA-B*5701 and take abacavir, or if they carry HLA-A*3101 and take carbamazepine.

• #17 Stevens-Johnson Syndrome: Practice Essentials, Background, Pathophysiology

  https://emedicine.medscape.com/article/1197450-overview

  Stevens-Johnson syndrome has been described worldwide in all races, although it may be more common in Whites. […] The proportion of females has been estimated to be 33-62%. […] In a large cohort, the mean age of patients with Stevens-Johnson syndrome was 25 years. In a smaller series, the mean age of patients with Stevens-Johnson syndrome was reported as 47 years. However, cases have been reported in children as young as 3 months and adults as old as 78 years.

• #18 Stevens–Johnson Syndrome (SJS) and Toxic Epidermal Necrolysis (TEN) | SpringerLink

  https://link.springer.com/10.1007/978-3-319-74698-2_195-1?fromPaywallRec=true

  The incidence of SJS and TEN is about one or two cases per one million people. TEN and SJS have been known to occur in patients of all ages, but the highest incidence is in adults greater than 40 years old. Incidence is about equal in men and women. Most cases tend to occur in winter or early spring, which could correlate with antibiotic prescriptions. […] An observational study of patients with SJS and TEN in the UK indicated patients of African or Asian ancestry had twice the chance of developing SJS and TEN compared with white patients; however, the general applicability of the study is limited by the low sample size. Higher incidence in patients with epilepsy, gout, and autoimmune diseases was thought to be largely attributable to associated medications rather than to the diseases themselves. No association was found between SJS and TEN and tobacco use, alcohol use, or obesity.

• #19 The Epidemiology of Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis in the UK

  https://edoc.unibas.ch/59044/

  Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are rare but life-threatening mucocutaneous diseases. […] We conducted a large observational study on the epidemiology of SJS/TEN using data from the UK-based Clinical Practice Research Datalink. Among 551 validated SJS/TEN patients, we calculated an incidence rate of 5.76 SJS/TEN cases per million person-years between 1995 and 2013, which was consistent throughout the study period and was highest in patients aged 1-10 years and 80 years or older. […] This large and longitudinal observational study on the epidemiology of SJS/TEN contributes to the understanding of this still underinvestigated severe skin disease in a European and largely white study population.

• #20 SJS /TEN ( Stephen Johnson Synd.) / (Toxic Epidermolysis Necrosis )

  https://emed.ie/Dermatology/SJS-TEN.php

  SJS: 1-2 million annually in US […] TEN: 0.4-1.2 M annually in US […] All ages races (x100 in HIV) […] in the elderly, F M = 2:1 […] HLA-B*1502, HLA-B*5801 (allopurinol-induced)

• #21 Toxic Epidermal Necrolysis (TEN): Background, Pathophysiology, Etiology

  https://emedicine.medscape.com/article/229698-overview

  In the United States, the annual frequency of TEN is reported to be 0.22-1.23 cases per 100,000 population. In the HIV-positive population, the incidence of TEN increases to 1 case per thousand per year. […] Worldwide, the average annual incidence of TEN is 0.4-1.3 cases per million population. […] In 1992, the cumulative incidence of TEN and SJS in Germany was 1.9 cases per million population. A French survey of dermatologists and health care facilities reported an annual incidence of 1 case per million population.

• #22 Stevens-Johnson syndrome epidemiology and demographics – wikidoc

  https://www.wikidoc.org/index.php/Stevens-Johnson_syndrome_epidemiology_and_demographics

  SJS is a rare condition, with a reported incidence of around 2-7 per 1000,000 people per year and a mean mortality rate of 4.8 percent. It is seen in patients of all age groups but particularly in children and young adults. Females are more commonly affected than males […] The incidence of SJS is approximately 2-7 per 1000,000 individuals per year. […] The incidences of SJS, SJS-TEN, and TEN were found to be a mean 5.3, 0.8, and 0.4 cases per million children per year in the US […] The mean estimated incidences of SJS, SJS/TEN, and TEN were found to be 9.2, 1.6, and 1.9 per million adults per year in the US […] Between years 2009-2012, the Mean adjusted mortality was 4.8% for SJS, 19.4% for SJS/TEN, and 14.8% for TEN […] Patients of all age groups may develop SJS. […] The incidence of SJS might be higher in Non White/Multiracial people as compared to White. […] Women are more commonly affected by SJS than males.

• #23 A Case of Stevens-Johnson Syndrome After Exposure to Valproic Acid

  https://www.psychiatrist.com/pcc/stevens-johnson-syndrome-after-exposure-to-valproic-acid/

  Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are acute life-threatening conditions that present with skin erosions and extensive detachment of the epidermis that is typically caused by drug exposure within weeks to months. […] Among anticonvulsants, phenytoin, lamotrigine, and carbamazepine are most often associated with SJS/TEN, while valproic acid is viewed as low risk. […] However, the risk of valproic acid with regard to SJS/TEN may be underestimated in current clinical practice. […] Further research is needed to define the risk for SJS/TEN with valproic acid when used in the absence of other anticonvulsants. The risk for SJS/TEN with valproic acid monotherapy may be underestimated in clinical practice. […] Physician knowledge regarding risk can guide clinical decision-making and facilitate early diagnosis and treatment of SJS/TEN. […] Lastly, the risk of anticonvulsant-induced SJS/TEN has been associated with genetic and ethnic background. […] Additional epidemiology and genetic studies are needed.

• #24 Stevens-Johnson Syndrome Associated with Drugs and Vaccines in Children: A Case-Control Study | PLOS One

  https://journals.plos.org/plosone/article?id=10.1371/journal.pone.0068231

  Stevens-Johnson Syndrome (SJS) is one of the most severe muco-cutaneous diseases and its occurrence is often attributed to drug use. The aim of the present study is to quantify the risk of SJS in association with drug and vaccine use in children. […] Since 1999 an active surveillance aimed at ascertaining the role of drugs and vaccines in the occurrence of specific acute conditions responsible for the hospitalization of paediatric patients has been conducted in Italy. […] Our study provides additional evidence on the etiologic role of drugs and vaccines in the occurrence of SJS in children. […] An increased risk of twenty-seven times was estimated for antiepileptic drugs. A three times increased risk was observed for antibiotics, even though the limited power of the study does not allow to differentiate between the three main classes: penicillins, cephalosporins and macrolides. Among the other drugs, a statistically significant increased risk was observed for paracetamol and corticosteroids, with OR estimates ranging between 3.2 and 4.2.

• #25 Stevens-Johnson syndrome | Radiology Reference Article | Radiopaedia.org

  https://radiopaedia.org/articles/stevens-johnson-syndrome-1?embed_domain=hackmd.io%252525252F%2525252540yipuafecsl2jsu8smr5njq%252525252Fbnjhjgjghjghjghfavicon.icofavicon.icofavicon.icofavicon.ico&lang=gb

  Stevens-Johnson syndrome is very rare, with an incidence of 1-2 per 1,000,000. […] Stevens-Johnson syndrome is thought to be triggered by preceding viral infection or more commonly medication use (e.g. NSAIDs, antibiotics, antiseizure medications).

• #26 Stevens-Johnson syndrome overview – wikidoc

  https://www.wikidoc.org/index.php/Stevens-Johnson_syndrome_overview

  SJS is a rare condition, with a reported incidence of around 2-7 per 1000,000 people per year and a mean mortality rate of 4.8 percent. It is seen in patients of all age groups but particularly in children and young adults. Females are more commonly affected than males […] Stevens-Johnson Syndrome is caused by drug reactions in the majority of the cases although it is also seen in certain viral and bacterial infections, and certain malignancies. Most commonly implicated drugs include sulfa drugs, Allopurinol, anti-epileptics (phenobarbital, carbamazepine, lamotrigine), antibiotics (for example Penicillin), and NSAIDs. Infections that are associated with SJS may include HIV, HSV, Mumps, Mycoplasma, Mycobacteria, Hepatitis and Streptococcal Infection.

• #27 Stephens-Johnson Syndrome – SJS – almostadoctor

  https://almostadoctor.co.uk/encyclopedia/stephens-johnson-syndrome-sjs

  Incidence of about 1-2 in a million per year […] 100x more common in patients with HIV […] Genetic factors predispose an individual to getting SJS / TEN […] More than 200 medications are known to cause SJS / TEN […] About 20% of cases are due to infection, or rarely vaccination.

• #28

  https://journals.lww.com/pidj/fulltext/2001/08000/stevens_johnson_syndrome_after_vaccination__in.30.aspx

  We thank Dr. Mortimer for his comments on our paper describing six reports of patients with Stevens-Johnson syndrome (SJS) after vaccination without evident alternative etiologies. […] Our report fits into the widely accepted scientific method for advancing medical knowledge by proposing a hypothesis in case reports, refining the hypothesis through case series and finally testing the hypothesis with controlled studies. […] The value of case series in vaccine safety surveillance is exemplified by the role played by 15 reports of intussusception after rotavirus vaccine to the Vaccine Adverse Event Reporting System. […] These additional cases support the need for further studies evaluating the possibility that vaccination causes some cases of SJS. […] We believe it is imperative to monitor case reports carefully to identify the most plausible and clinically significant potential vaccine adverse effects to promote public health and advance the science of vaccine safety.

• #29 Epidemiology of Stevens-Johnson syndrome and toxic epidermal necrolysis in the United States and factors predictive of outcome

  https://pmc.ncbi.nlm.nih.gov/articles/PMC10413346/

  The hospitalization rate of SJS/TEN has gradually declined over time, but the mortality rate due to SJS/TEN has remained stable between 2010 and 2020. […] We identified several risk factors associated with increased mortality including increasing age, racial/ethnic minority identification, chronic kidney disease, pneumonia, sepsis, and malignant neoplasm.

• #30 Stevens–Johnson syndrome and toxic epidermal necrolysis in Hong Kong | HKMJ

  https://www.hkmj.org/abstracts/v30n2/102.htm

  The estimated annual incidence of TEN alone and combined annual incidence of SJS, SJS-TEN overlap, and TEN were 1.36 and 5.07 cases per million, respectively; these incidences are comparable with findings from studies in other countries. […] The overall mortality rate was 21.6%; in 81.5% of these cases, the patient died of fulminant sepsis or multiorgan failure. […] In addition to high mortality, SJS/TEN were associated with high rates of burns unit/intensive care unit admission (32%) and prolonged length of stay (mean=23.9 days) […] This is the first large study in Hong Kong to provide data regarding the epidemiology, disease characteristics and clinical course, aetiology, treatment regimen, and mortality of SJS/TEN.

• #31

  https://www.jpad.com.pk/index.php/jpad/article/view/254?articlesBySameAuthorPage=3

  Mortality in cases of SJS/TEN showed significant association with wound infection, area of skin involvement, total morbidity score and respiratory system involvement. […] Mortality was found to be 13.3% and was significantly associated with infection, total morbidity score, area of epidermal involvement and respiratory system involvement.

• #32 A systematic review of the drug-induced Stevens-Johnson syndrome and toxic epidermal necrolysis in Indian population – Indian Journal of Dermatology, Venereology and Leprology

  https://ijdvl.com/a-systematic-review-of-the-drug-induced-stevens-johnson-syndrome-and-toxic-epidermal-necrolysis-in-indian-population/

  Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are rare severe cutaneous drug reactions. No large scale epidemiological data are available for this disorder in India. […] Incidence of SJS and TEN is 2.6-7.1 persons per million populations per year in United States. It is 1.1 and 0.93 per million per year for SJS and TEN respectively in Germany. Drugs are most commonly implicated for causing 77-95% of cases. […] The major causative drugs were antimicrobials (37.27%), anti-epileptics (35.73%) and non-steroidal anti-inflammatory drugs (15.93%). […] Higher mortality was observed for TEN as compared to SJS (odd ratio-7.19; 95% confidence interval (CI) 1.62-31.92; p= 0.0023). […] Total 62.96% of patients showed systemic complications. Most common complications were ocular (40.29%) and septicemia (17.65%). […] The presence of a severe systemic illness before the onset of SJS/TEN was a bad prognostic factor. […] Higher mortality, duration of stay and management cost are observed in TEN as compared to SJS that is in accordance with the studies abroad.

• #33 Stevens–Johnson Syndrome and Toxic Epidermal Necrolysis: A Review of Diagnosis and Management

  https://www.mdpi.com/1648-9144/57/9/895

  The management of SJS/TEN is multifaceted and begins with identification and cessation of the causative agent. […] Prognostication is also an important step in the management of SJS/TEN, as it can guide management and placement in an intensive care or burn unit. The severity-of-illness score for Toxic Epidermal Necrolysis (SCORTEN) scale is the most widely used tool for determining prognosis in patients with SJS/TEN. […] The goal of this article is to review the most recent updates in both diagnosis and management of SJS/TEN in order to educate dermatologists and other physicians who are managing the acute care of patients with SJS/TEN.

• #34 Clinical characteristics and treatment outcome of Stevens-Johnson syndrome and toxic epidermal necrolysis

  https://www.spandidos-publications.com/10.3892/etm.2015.2549

  Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are erythematous skin lesions with blister formation accompanied by mucosal involvement. These conditions are considered to be lifethreatening illnesses. […] The aim of the present study was to review and analyze the clinical manifestations, drug implications, treatment and outcome of patients with SJS and/or TEN who had been hospitalized in a tertiary care center. […] The mortality rates of these skin eruptions have been reported to range from 16 to 25%. […] The SCORTEN scoring system was used to grade the severity of these diseases. […] The mortality rate in the presented study was 6.9%, which was lower than the rates in previous studies. […] The severe forms of mucocutaneous eruptions, SJS and/or TEN, are mostly associated with adverse drug reactions. With early recognition and selected treatments, the mortality rate could be reduced. Improved understanding of clinical presentation and risk factors should help physicians to improve the care of high-risk individuals at an earlier stage. Patient age and the area of mucocutaneous involvement have been identified as significant factors associated with mortality.

• #35 Stevens-Johnson Syndrome – EyeWiki

  https://eyewiki.org/Stevens-Johnson_Syndrome

  Stevens-Johnson syndrome (SJS) is a dermatologic emergency, characterized by the presence of epidermal and mucosal bullous lesions involving less than 10% of the total body surface area (TBSA). It is a rare disease process with an estimated incidence of 2 to 7 cases per million per year. […] During the acute phase of SJS-TEN, 80% of patients will have ophthalmic involvement. […] Of note, chronic ocular changes secondary to SJS-TEN develop in 21-29% of pediatric cases and 27-59% of adult survivors. […] It is recommended that an ophthalmologist be consulted early in the course of suspected cases of SJS to initiate interventions that will lessen the likelihood of developing chronic ocular complications of SJS/TEN. […] The incidence of SJS was found to be 100 times higher in individuals infected with HIV relative to the general population. […] The overall incidence of chronic ocular surface changes is 21-29% in pediatric cases and 27-59% in adult SJS survivors.

• #36 Journal of Medical Internet Research

  https://www.jmir.org/

  Recruitment of Research Participants Improving Recruitment Through Social Media and Web-Based Advertising to Evaluate the Genetic Risk and Long-Term Complications in Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis: Community-Based Survey […] Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are genetically mediated life-threatening reactions that in adults are usually caused by a medication. These genetic associations promise an opportunity for pre-prescription screening, prevention, and understanding influences at a population level. Importantly, older adults disproportionally face more severe SJS/TEN reactions and higher mortality rates. However, the study of genetic risk and long-term sequelae of SJS/TEN across racially diverse populations and age groups, is hampered by many factors, including rarity, social disparities, and trust in healthcare and providers, which impact access to hospital and clinic-based research studies.

• #37 Stevens–Johnson Syndrome and Toxic Epidermal Necrolysis: Analysis of the Russian Database of Spontaneous Reports

  https://www.mdpi.com/1424-8247/17/6/675

  The total number of relevant spontaneous reports (SRs) on SJS and TEN registered in the AIS database was 170, which was 1.08% of the sample based on SOC “Skin and subcutaneous tissue disorders”, and 0.58% of the sample based on SOC “Immune system disorders”. […] The gained data indirectly indicate the rare development of SJS and TEN in the general population of Russia. […] The analysis of the SRs of the elderly with fatal outcomes revealed a mean age of 72.0 ± 9.1 (min=62, max=84) years, females were 60% (n = 3), and males were 40% (n = 2). […] The analysis of ATC level 1 groups involved in SJS and TEN in the elderly, children, and in the general population made it clear that age is associated with a different structure of leading causative agents. […] The limitations of our study are typical limitations specific to the spontaneous reporting method and include the following: underreporting, variable reporting rate considering different settings and time periods, the low quality of some spontaneous reports, and inability to establish the frequency of ADRs, since the total size of the population of drug users in unknown.

• #38 SciELO Brazil – Stevens-Johnson syndrome and toxic epidermal necrolysis: epidemiological and clinical outcomes analysis in public hospitals* Stevens-Johnson syndrome and toxic epidermal necrolysis: epidemiological and clinical outcomes analysis in public

  https://www.scielo.br/j/abd/a/cVdhgzbpkbXkBrVv5TWdQSN/

  Predominant drugs suspected of triggering SJS and TEN were anticonvulsants, followed by analgesics and antibiotics. […] In this study, we observed that SCORTEN is not used as a prognostic factor in evaluation of patients affected by SJS and NET, since there are no specific records in medical charts of the referral hospital. […] Limitations of this study lie in the fact that there is fragility in the registry of hospitalization of patients, both in SIH and in medical records of the referral hospital. Such fragility makes it difficult to construct a reliable and complete database on patients affected by such processes, which could be used to create protocols and standards in the treatment and prevention of these diseases, serving as a guide for professionals involved in the care of these patients.

• #39 Stevens–Johnson Syndrome and Toxic Epidermal Necrolysis: Analysis of the Russian Database of Spontaneous Reports

  https://www.mdpi.com/1424-8247/17/6/675

  Stevens–Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are extremely severe cutaneous adverse drug reactions which are relatively rare in routine clinical practice. […] An analysis of a national pharmacovigilance database may be the most effective method of obtaining information on SJS and TEN. […] The incidence of SJS and TEN is low in the general population, though the lethality is high. […] The assessment of SJS and TEN epidemiology in the USA based on the total number of hospitalizations revealed 51,040 (0.1%) hospitalizations for SJS/TEN, with a higher prevalence of SJS (73%, n = 37,283). […] Pharmacovigilance databases may be a valuable instrument to study SJS and TEN prevalence and the structure of suspected drugs. […] The objective of our study was to assess SJS and TEN characteristics in the general population of Russia, in the children, and in the elderly using data from the Russian National Pharmacovigilance database.

• #40 Erythema multiforme, Stevens Johnson syndrome, and toxic epidermal necrolysis reported after vaccination, 1999–2017

  https://stacks.cdc.gov/view/cdc/85009

  Background: Since the last review of vaccine safety surveillance data for erythema multiforme (EM), Stevens Johnson syndrome (SJS), SJS/TEN, and toxic epidermal necrolysis (TEN) (EM/SJS/TEN), over 37 new vaccines have been introduced in the United States. We sought to describe reported EM/SJS/TEN after vaccines during 19992017. […] We identified U.S. reports of EM/SJS/TEN received by the Vaccine Adverse Event Reporting System (VAERS) during 19992017. […] Of 466,027 reports to VAERS during 19992017, we identified 984 reports of EM, 89 reports of SJS, 6 reports of SJS/TEN, and 7 reports of TEN. […] Overall, 55% of reports described males, 48% described children aged […] EM/SJS/TEN were rarely reported after vaccination; data mining identified a known association between EM and smallpox vaccine.

• #41

  https://www.irten.org/en/about-sjs-ten/epidemiology-causes-risk-factors

  Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are both rare (estimated incidence rates for TEN of 0.4 to 1.2 cases per million person-years, and SJS of 1 to 6 cases per million person-years), but extremely severe and potentially life-threatening dermatological conditions. […] The IRTEN prospective registry aims to provide more precise information concerning the epidemiology, causes (medications) and risk factors including analysis of regional/ethnic variability.

• #42 Effectiveness of early treatment with plasma exchange in patients with Stevens–Johnson syndrome and toxic epidermal necrolysis | Scientific Reports

  https://www.nature.com/articles/s41598-024-53653-5

  Stevens-Johnson syndrome and toxic epidermal necrolysis (SJS/TEN) are potentially fatal medical conditions that lack established treatment. […] Therapeutic plasma exchange (PE) is another treatment option but evidence of its effectiveness is limited to case reports and small case series. […] The objective of this study was to assess the effectiveness of PE in patients with SJS/TEN. […] The effectiveness of initiating PE immediately on hospital admission in patients with severe SJS/TEN was evaluated using a nationwide database and adjusted using a doubly robust estimation method to minimise confounding. The results showed no benefit of PE for reducing in-hospital mortality or the length of hospital stay. […] This study, with appropriate adjustments for concurrent treatment, provided no evidence that PE reduced mortality or shortened the length of hospital stay. […] In contrast to previous studies, this study did not find any evidence of a benefit of early PE in patients with severe SJS/TEN.

• #43 Research Directions in Genetically-Mediated Stevens-Johnson Syndrome/Toxic Epidermal Necrolysis

  https://www.genome.gov/27560487/research-directions-in-geneticallymediated-stevensjohnson-syndrometoxic-epidermal-necrolysis

  Review the current state of knowledge of the surveillance, pathogenesis and treatment of Stevens-Johnson Syndrome/Toxic Epidermal Necrolysis (SJS/TEN). […] Pharmacosurveillance for SJS/TEN in the United States. […] Discussion of pharmacosurveillance research opportunities.

• #44

  https://www.ijmscr.ijpbms.com/index.php/ijmscrs/article/view/527

  Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are severe mucocutaneous responses caused by drugs that are characterized by widespread necrosis and epidermal detachment. […] It is essential to know the risk factors and those agents involved in the development of this pathology in order to use them with caution in susceptible patients and to have a high index of suspicion.

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