Chun-Bing Chen1, Tsun-Hao Hsu2, Rosaline Chung-Yee Hui2, Chun-Wei Lu3, Wei-Ti Chen2, Pin-Hsuan Chiang2, Chuang-Wei Wang4, Shiow-Shuh Chuang5, Jui-Yung Yang5, Shih-Yi Yang5, Shu-Ying Chang5, Yen-Chang Hsiao5, Kuo-Chin Kao6, Han-Chung Hu6, Ting-Shu Wu7, Chao-Wei Hsu8, David Hui-Kang Ma9, Shin-Yi Chen10, Ya-Chung Tian11, Chi-Yuan Cheng12, Chi-Hua Chen12, Min-Hui Chi2, Ming-Ying Wu2, Ren-Feng Liu2, Chi-Hui Wang2, Ya-Ching Chang2, Jing-Yi Lin2, Hsin-Chun Ho2, Yang Yu-Wei Lin13, Chee Jen Chang14, Yu-Jr Lin14, Cheng-Lung Ku15, Shuen-Iu Hung16, Wen-Hung Chung17. 1. Department of Dermatology, Drug Hypersensitivity Clinical and Research Center, Chang Gung Memorial Hospital, Linkou, Taipei, Keelung, Taoyuan, Taiwan; College of Medicine, Chang Gung University, Taoyuan, Taiwan; Graduate Institute of Clinical Medical Sciences, College of Medicine, Chang Gung University, Kwei-Shan, Taoyuan, Taiwan; Chang Gung Immunology Consortium, Chang Gung Memorial Hospital, Linkou, Taoyuan, Taiwan; Cancer Vaccine and Immune Cell Therapy Core Laboratory, Chang Gung Memorial Hospital, Linkou, Taoyuan, Taiwan; Immune-Oncology Center of Excellence, Chang Gung Memorial Hospital, Linkou, Taoyuan, Taiwan; Department of Dermatology, Xiamen Chang Gung Hospital, Xiamen, China. 2. Department of Dermatology, Drug Hypersensitivity Clinical and Research Center, Chang Gung Memorial Hospital, Linkou, Taipei, Keelung, Taoyuan, Taiwan; College of Medicine, Chang Gung University, Taoyuan, Taiwan. 3. Department of Dermatology, Drug Hypersensitivity Clinical and Research Center, Chang Gung Memorial Hospital, Linkou, Taipei, Keelung, Taoyuan, Taiwan; College of Medicine, Chang Gung University, Taoyuan, Taiwan; Graduate Institute of Clinical Medical Sciences, College of Medicine, Chang Gung University, Kwei-Shan, Taoyuan, Taiwan; Chang Gung Immunology Consortium, Chang Gung Memorial Hospital, Linkou, Taoyuan, Taiwan; Immune-Oncology Center of Excellence, Chang Gung Memorial Hospital, Linkou, Taoyuan, Taiwan. 4. Department of Dermatology, Drug Hypersensitivity Clinical and Research Center, Chang Gung Memorial Hospital, Linkou, Taipei, Keelung, Taoyuan, Taiwan; College of Medicine, Chang Gung University, Taoyuan, Taiwan; Graduate Institute of Clinical Medical Sciences, College of Medicine, Chang Gung University, Kwei-Shan, Taoyuan, Taiwan; Chang Gung Immunology Consortium, Chang Gung Memorial Hospital, Linkou, Taoyuan, Taiwan; Cancer Vaccine and Immune Cell Therapy Core Laboratory, Chang Gung Memorial Hospital, Linkou, Taoyuan, Taiwan. 5. Department of Plastic and Reconstructive Surgery, The Burn Center, Chang Gung Memorial Hospital, Taoyuan, Taiwan. 6. Department of Thoracic Medicine, Chang Gung Memorial Hospital, Taoyuan, Taiwan; Clinical Informatics Department of Respiratory Therapy, College of Medicine, Chang Gung University, Taoyuan, Taiwan. 7. Division of Infectious Diseases, Department of Internal Medicine, Chang Gung Memorial Hospital, Chang Gung University College of Medicine, Kweishan, Taoyuan, Taiwan. 8. Department of Hepatogastroenterology, Liver Research Center, Chang Gung Memorial Hospital, Taoyuan, Taiwan. 9. Department of Ophthalmology, Chang Gung Memorial Hospital, Linkou, Taoyuan, Taiwan. 10. Department of Ophthalmology, Chang Gung Memorial Hospital, Keelung, Taiwan. 11. Department of Nephrology, Kidney Research Center, Chang Gung Memorial Hospital, Linkou, Taoyuan, Taiwan. 12. Department of Pharmacy, Chang Gung Memorial Hospital, Linkou, Taoyuan, Taiwan. 13. Department of Dermatology, Drug Hypersensitivity Clinical and Research Center, Chang Gung Memorial Hospital, Linkou, Taipei, Keelung, Taoyuan, Taiwan. 14. College of Medicine, Chang Gung University, Taoyuan, Taiwan; Research Services Center for Health Information, Chang Gung University, Taoyuan, Taiwan; Clinical Informatics and Medical Statistics Research Center, Chang Gung University, Taoyuan, Taiwan. 15. Laboratory of Human Immunology and Infectious Disease, Graduate Institute of Clinical Medical Sciences, Chang Gung University, Taoyuan, Taiwan; Department of Nephrology, Chang Gung Memorial Hospital, Taoyuan, Taiwan. 16. College of Medicine, Chang Gung University, Taoyuan, Taiwan; Cancer Vaccine and Immune Cell Therapy Core Laboratory, Chang Gung Memorial Hospital, Linkou, Taoyuan, Taiwan. 17. Department of Dermatology, Drug Hypersensitivity Clinical and Research Center, Chang Gung Memorial Hospital, Linkou, Taipei, Keelung, Taoyuan, Taiwan; College of Medicine, Chang Gung University, Taoyuan, Taiwan; Chang Gung Immunology Consortium, Chang Gung Memorial Hospital, Linkou, Taoyuan, Taiwan; Cancer Vaccine and Immune Cell Therapy Core Laboratory, Chang Gung Memorial Hospital, Linkou, Taoyuan, Taiwan; Immune-Oncology Center of Excellence, Chang Gung Memorial Hospital, Linkou, Taoyuan, Taiwan; Department of Dermatology, Xiamen Chang Gung Hospital, Xiamen, China; Department of Dermatology, Beijing Tsinghua Chang Gung Hospital, School of Clinical Medicine, Tsinghua University, Beijing, China; School of Medicine, Shanghai Jiao Tong University, Shanghai, China. Electronic address: wenhungchung@yahoo.com.
Abstract
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.
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.