New Grading System for the Evaluation of Chronic Ocular Manifestations in Patients with Stevens–Johnson Syndrome

Sotozono, Chie; Ang, Leonard P. K.; Koizumi, Noriko; Higashihara, Hisayo; Ueta, Mayumi; Inatomi, Tsutomu; Yokoi, Norihiko; Kaido, Minako; Doğru, Murat; Shimazaki, Jun; Yamada, Masakazu; Kinoshita, Shigeru

doi:10.1016/j.ophtha.2006.10.029

Cited by 259 publications

(205 citation statements)

References 26 publications

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“…7 SOC patients were defined as those with ocular sequelae such as severe dry eye, trichiasis, symblepharon and conjunctival invasion into the cornea in the chronic stage, 5 and as patients who manifested severe conjunctivitis with pseudomembranes and epithelial defects on the ocular surface (cornea and/or conjunctiva) in the acute stage. 14 We have classified the patients who had taken CMs such as non-steroidal anti-inflammatory drugs and multi-ingredient cold medications for a few-several days before the disease onset for common-cold symptoms as CM-SJS/TEN.…”

Section: Materials and Methods Patientsmentioning

confidence: 99%

“…4 Survivors often suffer severe sequelae such as vision loss due to severe ocular complications (SOC). 5 About 40% of SJS/TEN patients diagnosed by dermatologists develop SOC. 2 Among SJS and TEN patients, especially those with SJS/TEN with SOC, cold medicines (CM), including multi-ingredient cold medications and non-steroidal anti-inflammatory drugs, were identified as causative.…”

Section: Stevens-johnson Syndrome (Sjs)mentioning

confidence: 99%

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Genome-wide association study using the ethnicity-specific Japonica array: identification of new susceptibility loci for cold medicine-related Stevens–Johnson syndrome with severe ocular complications

et al. 2017

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A genome-wide association study (GWAS) for cold medicine-related Stevens-Johnson syndrome (CM-SJS) with severe ocular complications (SOC) was performed in a Japanese population. A recently developed ethnicity-specific array with genome-wide imputation that was based on the whole-genome sequences of 1070 unrelated Japanese individuals was used. Validation analysis with additional samples from Japanese individuals and replication analysis using samples from Korean individuals identified two new susceptibility loci on chromosomes 15 and 16. This study might suggest the usefulness of GWAS using the ethnicity-specific array and genome-wide imputation based on large-scale whole-genome sequences. Our findings contribute to the understanding of genetic predisposition to CM-SJS with SOC.

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Section: Materials and Methods Patientsmentioning

confidence: 99%

Section: Stevens-johnson Syndrome (Sjs)mentioning

confidence: 99%

Genome-wide association study using the ethnicity-specific Japonica array: identification of new susceptibility loci for cold medicine-related Stevens–Johnson syndrome with severe ocular complications

et al. 2017

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“…In the chronic stage, ocular surface complications such as conjunctival invasion into the cornea due to corneal epithelial stem cell deficiency, symblepharon, ankyloblepharon and, in some instances, keratinisation of the ocular surface persist despite healing of the skin lesions (see Figure 1). 9 We documented elsewhere that more than 95% of patients with SJS/TEN with ocular surface complications had lost their fingernails in the acute or subacute stage, and that some continued to have transformed nails even after healing of their skin lesions (see Figure 2). 10 Our diagnosis of SJS/TEN (SJS in a broad sense) was based on a confirmed history of acute-onset high fever, serious mucocutaneous illness with skin eruptions and involvement of at least two mucosal sites, including the ocular surface.…”

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confidence: 93%

Ethnic Differences in the Association Between Human Leukocyte Antigen and Stevens-Johnson Syndrome

Ueta¹

2009

European Ophthalmic Review

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The HLA-B12 antigen is significantly increased in Caucasian patients with Stevens-Johnson syndrome (SJS) with ocular complications, while HLA-A*0206 is strongly associated with Japanese patients with SJS/toxic epidermal necrolysis (TEN) with ocular complications. There are strong ethnic differences in the association between HLA and SJS/TEN. Regarding the association between HLA and drug-induced severe cutaneous adverse reactions (SCAR), including SJS and TEN, the strong allopurinol-specific association between HLA-B*5801 and allopurinol-induced SCAR may be a universal phenomenon, since it has been identified in all Han Chinese, Caucasian and Japanese patients. In contrast, the carbamazepine-specific association between HLA-B*1502 and carbamazepine-induced SJS may be specific to certain ethnic groups, as it has been identified in Han Chinese but not in Caucasian and Japanese patients. Dermatologists have reported that allopurinol and anticonvulsant drugs such as carbamazepine are commonly associated with SJS/TEN, while many of the author's patients developed SJS after receiving treatment for the common cold with antibiotics, cold remedies and/or non-steroidal antiinflammatory drugs (NSAIDs). This article posits that the SJS/TEN patients seen by dermatologists are not always the same as the SJS/TEN patients consulting opthalmologists. KeywordsStevens-Johnson syndrome (SJS), toxic epidermal necrolysis (TEN), ocular surface complications, human leukocyte antigen (HLA), ethnic differences, severe cutaneous adverse reactions (SCAR), drug, carbamazepine, allopurinol In contrast, dermatologists claimed that more than 100 drugs were involved in eliciting SJS and its severe variant, toxic epidermal necrolysis (TEN). 4 They cited life-threatening severe adverse drug reactions characterised by high fever, rapidly developing blistering exanthema of macules and target-like lesions accompanied by mucosal involvement and skin detachment. 4,5Formerly, erythema multiforme (EM), SJS and TEN were accepted as being part of a single EM spectrum; however, a retrospective analysis of the type and distribution of the skin lesions and the extent of epidermal detachment identified EM major and SJS/TEN as two distinct clinical entities that differed with respect to their histopathological changes and aetiology. 6 The annual incidence of SJS and TEN has been estimated as 0.4 to one and one to six cases per million persons, respectively; 6,7 the reported mortality rate is 3 and 27%, respectively. 8 Although rare, these reactions carry high morbidity and mortality rates and often result in severe and definitive sequelae such as vision loss. The pathobiological mechanisms underlying the onset of SJS/TEN have not been fully established. The extreme rarity of cutaneous and ocular surface reactions to drug therapies led us to suspect individual susceptibility.In the acute stage, SJS/TEN patients manifest severe conjunctivitis and corneal/conjunctival epithelial defects with vesiculobullous skin lesions. In the chronic stage, ocular surf...

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“…Particularly, ocular complications ranging from mild symptoms to very severe symptoms, such as corneal melting and perforation, which sometimes cause blindness, occur in more than 50% of patients. 6) Therefore, the estimation of severity of cutaneous and ocular symptoms and the therapeutic intervention in early stage are important to avoid lowering the patient's quality-of-life.…”

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confidence: 99%

Effect of Infectious Diseases on the Pathogenesis of Stevens–Johnson Syndrome and Toxic Epidermal Necrolysis

Okamoto‐Uchida¹,

Nakamura²,

Sai³

et al. 2017

Biological & Pharmaceutical Bulletin

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Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are severe cutaneous adverse drug reactions. Recent studies have revealed that the prevalence of SJS/TEN is associated with genetic backgrounds, such as polymorphisms in human leukocyte antigens (HLAs). However, non-genetic factors contributing to the etiology of SJS/TEN are largely unknown. This study aimed to assess the involvement of concurrent infection on the pathological states of SJS/TEN, examining the severity of cutaneous symptoms and ocular involvement as well as the time to onset in drug-induced SJS/TEN patients. We recruited 257 Japanese SJS/TEN patients from June 2006 to September 2013 through a nationwide case collection network and participating hospitals and reviewed the clinical information including patient backgrounds, primary disease and medication status. Association between infection and pathological states of SJS/TEN was assessed using univariate and multivariate analyses. The concurrent infectious group of SJS/TEN patients showed a significantly higher rate of exhibiting severer dermatological and ophthalmological phenotypes and an earlier onset of SJS/TEN than the non-infectious group. Our results suggest that the infection could be a risk factor to cause severer symptoms and earlier onset of SJS/TEN. Key words Stevens-Johnson syndrome; toxic epidermal necrolysis; infection; epidemiological study; heterologous immunity Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are diseases within the same spectrum of immune-mediated cutaneous adverse drug reactions characterized by the detachment of the epidermis and erosion of the mucous membrane.1) These disorders are categorized according to the extent of body surface area involved.2,3) Although SJS and TEN are very rare, the associated mortality rates are very high; recently, the mortality rate was reported to be 34% for SJS/TEN at 1 year after onset in Europe,4) and was 3 and 19% for SJS and TEN, respectively, in Japan.5) Depending on the mucosal involvements in the acute stage of these diseases, various long-lasting sequelae and complications may develop. Particularly, ocular complications ranging from mild symptoms to very severe symptoms, such as corneal melting and perforation, which sometimes cause blindness, occur in more than 50% of patients. 6) Therefore, the estimation of severity of cutaneous and ocular symptoms and the therapeutic intervention in early stage are important to avoid lowering the patient's quality-of-life.The pathological mechanisms of SJS/TEN remain largely unclear. In the cutaneous lesions of SJS/TEN, soluble Fas ligand (FasL) and granulysin as well as T-helper 1 (Th1) and T-helper 2 (Th2) cytokines produced by activated T cells were strongly expressed, which is considered to cause keratinocyte death. 7,8) Recent studies have revealed that the prevalence of SJS/TEN is associated with genetic backgrounds such as polymorphisms in human leukocyte antigens (HLAs) and a metabolic enzyme, CYP.9-16) However, not all patients with these h...

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New Grading System for the Evaluation of Chronic Ocular Manifestations in Patients with Stevens–Johnson Syndrome

Cited by 259 publications

References 26 publications

Genome-wide association study using the ethnicity-specific Japonica array: identification of new susceptibility loci for cold medicine-related Stevens–Johnson syndrome with severe ocular complications

Genome-wide association study using the ethnicity-specific Japonica array: identification of new susceptibility loci for cold medicine-related Stevens–Johnson syndrome with severe ocular complications

Ethnic Differences in the Association Between Human Leukocyte Antigen and Stevens-Johnson Syndrome

Effect of Infectious Diseases on the Pathogenesis of Stevens–Johnson Syndrome and Toxic Epidermal Necrolysis

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