E. Sato, Hisatomi Arima, Kotaro Ito, Mayuko Iwata, Shinichi Imafuku
It remains unclear which therapy contributes to atopic dermatitis (AD) remission and to what extent. We aimed to clarify which therapy contributes to the treatment of AD by investigating the time-to-remission and remission hazard ratios for each therapy using real-world data.This retrospective cohort study included 110 patients diagnosed with AD after their first visit to the Department of Dermatology at Fukuoka University Hospital between 2016 and 2022. The patients were categorized into six treatment groups: 1) topical treatment alone or topical treatment plus 2) ultraviolet light, 3) oral steroids, 4) oral cyclosporine, 5) dupilumab, and 6) oral Janus kinase inhibitors (JAKi). The topical therapy alone group served as the control, and the hazard ratios for remission (Investigator’s Global Assessment [IGA] 0/1) were calculated.Forty patients achieved remission, while 70 did not (IGA ≥2) with the first treatment regimen. A multivariate Cox proportional hazards analysis adjusted for age, sex, and severity at the first visit (IGA) revealed that the hazard ratios for remission were 4.2 (95% confidence interval (C.I.): 1.28–13.83, p = 0.018) for the oral cyclosporine group, 5.05 (95% C.I.: 1.96–13, p = 0.001) for the dupilumab group, and 67.56 (95% C.I.: 12.28–371.68, p < .0001) for the oral JAKi group. The median time to remission was 3 months for JAKi, cyclosporine, and steroid was shorter than 6 months for dupilumab. No serious adverse events were observed.Oral therapy with small molecules requires a shorter duration to achieve remission. However, long-term safety and recurrence are important indicators.
{"title":"Comparative effectiveness of treatments on time to remission in atopic dermatitis: real-world insights","authors":"E. Sato, Hisatomi Arima, Kotaro Ito, Mayuko Iwata, Shinichi Imafuku","doi":"10.3389/jcia.2024.12974","DOIUrl":"https://doi.org/10.3389/jcia.2024.12974","url":null,"abstract":"It remains unclear which therapy contributes to atopic dermatitis (AD) remission and to what extent. We aimed to clarify which therapy contributes to the treatment of AD by investigating the time-to-remission and remission hazard ratios for each therapy using real-world data.This retrospective cohort study included 110 patients diagnosed with AD after their first visit to the Department of Dermatology at Fukuoka University Hospital between 2016 and 2022. The patients were categorized into six treatment groups: 1) topical treatment alone or topical treatment plus 2) ultraviolet light, 3) oral steroids, 4) oral cyclosporine, 5) dupilumab, and 6) oral Janus kinase inhibitors (JAKi). The topical therapy alone group served as the control, and the hazard ratios for remission (Investigator’s Global Assessment [IGA] 0/1) were calculated.Forty patients achieved remission, while 70 did not (IGA ≥2) with the first treatment regimen. A multivariate Cox proportional hazards analysis adjusted for age, sex, and severity at the first visit (IGA) revealed that the hazard ratios for remission were 4.2 (95% confidence interval (C.I.): 1.28–13.83, p = 0.018) for the oral cyclosporine group, 5.05 (95% C.I.: 1.96–13, p = 0.001) for the dupilumab group, and 67.56 (95% C.I.: 12.28–371.68, p < .0001) for the oral JAKi group. The median time to remission was 3 months for JAKi, cyclosporine, and steroid was shorter than 6 months for dupilumab. No serious adverse events were observed.Oral therapy with small molecules requires a shorter duration to achieve remission. However, long-term safety and recurrence are important indicators.","PeriodicalId":508694,"journal":{"name":"Journal of Cutaneous Immunology and Allergy","volume":"114 51","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-06-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141360851","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Hanako Miyahara, Noriko Kubota, M. Okune, Yoshiyuki Ishii, N. Okiyama, T. Nomura, Junichi Furuta
Acquired idiopathic generalized anhidrosis is a rare disease characterized by systemic anhidrosis or hypohidrosis without other systemic diseases. However, its etiology remains unclear. Autoimmune mechanisms seem to be involved in the development of acquired idiopathic generalized anhidrosis. Although steroid pulse therapy is the most commonly used therapy, it lacks a high level of evidence. On the other hand, pilocarpine, a muscarinic receptor agonist that stimulates exocrine glands, increases saliva and tear secretion as well as sweating. Here, we report treatment progresses of steroid pulse therapy and oral pilocarpine in our department. Between 2012 and 2021, we treated 10 patients of acquired idiopathic generalized anhidrosis. All patients were administered oral pilocarpine as the first therapy, three (30%) of whom had increased sweating. Minor side effects were observed, however, no serious side effects were observed. Five patients who did not respond to oral pilocarpine were subsequently treated with steroid pulse therapy to which four (80%) showed significant response. Two patients were subsequently administered oral pilocarpine as post-therapy, and remission was maintained for up to 81 months. Oral pilocarpine could be used to relieve symptoms in patients with acquired idiopathic generalized anhidrosis prior to steroid pulse therapy and as maintenance therapy after steroid pulse therapy.
{"title":"Case report: Ten cases of acquired idiopathic generalized anhidrosis treated with oral pilocarpine","authors":"Hanako Miyahara, Noriko Kubota, M. Okune, Yoshiyuki Ishii, N. Okiyama, T. Nomura, Junichi Furuta","doi":"10.3389/jcia.2024.12902","DOIUrl":"https://doi.org/10.3389/jcia.2024.12902","url":null,"abstract":"Acquired idiopathic generalized anhidrosis is a rare disease characterized by systemic anhidrosis or hypohidrosis without other systemic diseases. However, its etiology remains unclear. Autoimmune mechanisms seem to be involved in the development of acquired idiopathic generalized anhidrosis. Although steroid pulse therapy is the most commonly used therapy, it lacks a high level of evidence. On the other hand, pilocarpine, a muscarinic receptor agonist that stimulates exocrine glands, increases saliva and tear secretion as well as sweating. Here, we report treatment progresses of steroid pulse therapy and oral pilocarpine in our department. Between 2012 and 2021, we treated 10 patients of acquired idiopathic generalized anhidrosis. All patients were administered oral pilocarpine as the first therapy, three (30%) of whom had increased sweating. Minor side effects were observed, however, no serious side effects were observed. Five patients who did not respond to oral pilocarpine were subsequently treated with steroid pulse therapy to which four (80%) showed significant response. Two patients were subsequently administered oral pilocarpine as post-therapy, and remission was maintained for up to 81 months. Oral pilocarpine could be used to relieve symptoms in patients with acquired idiopathic generalized anhidrosis prior to steroid pulse therapy and as maintenance therapy after steroid pulse therapy.","PeriodicalId":508694,"journal":{"name":"Journal of Cutaneous Immunology and Allergy","volume":"1 3","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-06-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141267590","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Tamihiro Kawakami, Koichiro Nakamura, Y. Kirino, M. Takeno
{"title":"A proposal of a comprehensive mucocutaneous activity index for Behçet’s disease","authors":"Tamihiro Kawakami, Koichiro Nakamura, Y. Kirino, M. Takeno","doi":"10.3389/jcia.2024.12652","DOIUrl":"https://doi.org/10.3389/jcia.2024.12652","url":null,"abstract":"","PeriodicalId":508694,"journal":{"name":"Journal of Cutaneous Immunology and Allergy","volume":"15 58","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-05-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141016765","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Azusa Ida, Osamu Ishikawa, Akihiko Uchiyama, E. Akasaka
{"title":"A case of dominant dystrophic epidermolysis bullosa pruriginosa with a novel mutation in the hinge region of COL7A1","authors":"Azusa Ida, Osamu Ishikawa, Akihiko Uchiyama, E. Akasaka","doi":"10.3389/jcia.2024.12844","DOIUrl":"https://doi.org/10.3389/jcia.2024.12844","url":null,"abstract":"","PeriodicalId":508694,"journal":{"name":"Journal of Cutaneous Immunology and Allergy","volume":"33 2","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141057891","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Kentaro Kawate, H. Kasamatsu, Kentarou Nishimura, Yoriko Muneishi, Wataru Takashima, Haruka Koizumi, Shiro Iino, Kouji Hayashi, N. Oyama, Minoru Hasegawa
{"title":"A case of refractory peripheral neuropathy associated with eosinophilic granulomatosis with polyangiitis with successful tapering of systemic corticosteroid and reduced dosing frequency of high-dose intravenous immunoglobulin after combined use of mepolizumab","authors":"Kentaro Kawate, H. Kasamatsu, Kentarou Nishimura, Yoriko Muneishi, Wataru Takashima, Haruka Koizumi, Shiro Iino, Kouji Hayashi, N. Oyama, Minoru Hasegawa","doi":"10.3389/jcia.2024.12776","DOIUrl":"https://doi.org/10.3389/jcia.2024.12776","url":null,"abstract":"","PeriodicalId":508694,"journal":{"name":"Journal of Cutaneous Immunology and Allergy","volume":" 74","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-04-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140685122","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Akiko Sugiyama, Takehito Fukushima, Koki Okabe, K. Shimada, Kaoru Kojima, Yuko Shigeoka, Satoshi Honjo, Reiko Kishikawa
Background: Wheat-dependent exercise-induced anaphylaxis (WDEIA) is induced not by the intake of wheat-based food alone, but by the combination of exercise stress and such intake. Provocation tests have been reported to be useful for the diagnosis of this condition, but they are not always accurate. Furthermore, provocation tests are associated with the risk of anaphylactic shock, so safer testing is required.Subjects and methods: Thirty-three patients (mean age 35.5 ± 16.0 years) who underwent provocation tests from April 2019 to July 2023 were included in this study. We investigated associations of their provocation test results with their medical history, blood test results before the provocation tests, exercise load at the time of the test, and severity of the induced symptoms.Results: Symptoms were induced and the diagnosis of WDEIA was made in 28 cases, while 2 cases were confirmed not to have WDEIA. Overall, 25 of the 28 positive cases had symptoms induced by an exercise load greater than 70% of the heart rate calculated by the Karvonen formula, 7 of which required Non-Steroidal Anti-Inflammatory Drugs (NSAIDs) loading to induce symptoms. None of the patients with negative wheat-specific IgE titers and positive gluten and/or ω-5 gliadin ones required NSAIDs loading. No patients required the administration of adrenaline.Conclusion: Wheat-specific IgE as well as gluten and ω-5 gliadin antibody titers are associated with WDEIA symptom severity. Exercise loading with a target heart rate of 70%–90% of that calculated by the Karvonen formula is safe and useful for diagnosis this condition.
{"title":"Effective and safe provocation test for wheat-dependent exercise-induced anaphylaxis (WDEIA) in adults results of testing on 33 cases","authors":"Akiko Sugiyama, Takehito Fukushima, Koki Okabe, K. Shimada, Kaoru Kojima, Yuko Shigeoka, Satoshi Honjo, Reiko Kishikawa","doi":"10.3389/jcia.2024.12896","DOIUrl":"https://doi.org/10.3389/jcia.2024.12896","url":null,"abstract":"Background: Wheat-dependent exercise-induced anaphylaxis (WDEIA) is induced not by the intake of wheat-based food alone, but by the combination of exercise stress and such intake. Provocation tests have been reported to be useful for the diagnosis of this condition, but they are not always accurate. Furthermore, provocation tests are associated with the risk of anaphylactic shock, so safer testing is required.Subjects and methods: Thirty-three patients (mean age 35.5 ± 16.0 years) who underwent provocation tests from April 2019 to July 2023 were included in this study. We investigated associations of their provocation test results with their medical history, blood test results before the provocation tests, exercise load at the time of the test, and severity of the induced symptoms.Results: Symptoms were induced and the diagnosis of WDEIA was made in 28 cases, while 2 cases were confirmed not to have WDEIA. Overall, 25 of the 28 positive cases had symptoms induced by an exercise load greater than 70% of the heart rate calculated by the Karvonen formula, 7 of which required Non-Steroidal Anti-Inflammatory Drugs (NSAIDs) loading to induce symptoms. None of the patients with negative wheat-specific IgE titers and positive gluten and/or ω-5 gliadin ones required NSAIDs loading. No patients required the administration of adrenaline.Conclusion: Wheat-specific IgE as well as gluten and ω-5 gliadin antibody titers are associated with WDEIA symptom severity. Exercise loading with a target heart rate of 70%–90% of that calculated by the Karvonen formula is safe and useful for diagnosis this condition.","PeriodicalId":508694,"journal":{"name":"Journal of Cutaneous Immunology and Allergy","volume":" 20","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-04-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140687646","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Successful control of Morbihan disease with dapsone: a case report and literature review","authors":"Yuka Morita, Haruka Koizumi, Yuki Arisawa, Ryo Fukaura, Kyoko Sugawara","doi":"10.3389/jcia.2024.12849","DOIUrl":"https://doi.org/10.3389/jcia.2024.12849","url":null,"abstract":"","PeriodicalId":508694,"journal":{"name":"Journal of Cutaneous Immunology and Allergy","volume":"30 32","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-04-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140697021","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Background: The nature of itch sensation varies depending upon the patient and the disease. However, few studies have focused on verbal expressions describing itch of atopic dermatitis (AD) in quality.Objectives: To investigate itch quality in patients with AD compared with that of urticaria.Methods: We conducted an online questionnaire survey describing itch experiences in June 2021. Participants were Japanese patients who had visited hospitals for their consultations and treatments of AD or urticaria in the last 6 months, and 295 and 290 responses, respectively, to questions using 12 terms describing itch quality were analyzed.Results: The most suitable expression describing intense itch that patients could not help scratching differed between the diseases, where most AD patients selected “muzumuzu” (a mimetic word for creepy–crawly itch) (27%) or “painful itch” (20%), and most urticaria patients selected “muzumuzu” (24%) or “itch like mosquito bites” (22%). The most suitable expressions describing itch that would make patients happiest if improved was “painful itch” (27%) in AD patients, significantly higher than urticaria patients (19%). More AD patients (55%) responded that they sometimes felt itch even after the skin symptoms had subsided than urticaria patients (41%). The most suitable expression of remnant itch selected was “muzumuzu” for AD (58/161 patients, 36%) and urticaria (29/120 patients, 24%).Conclusion: The quality of itch sensations can be classified not only between diseases but also during the clinical course of each disease. Significant expressions that patients with AD use to describe itch sensations could promote more appropriate treatment for itch.
{"title":"Verbal expressions describing itch quality in atopic dermatitis and urticaria: an online questionnaire survey in Japan","authors":"Yukihiro Ohya, Toshiya Ebata, Yusei Ohshima, Tsugunobu Andoh, Mitsutoshi Tominaga, Yoko Kataoka, Yoshinori Fukui, Nobuyuki Ebihara, Shunji Hasegawa, Shigetoshi Kobayashi, Yutaka Morisawa, Norihiro Inoue, Masami Narita, Sakae Kaneko, Ken Igawa, Takeshi Nakahara, Y. Ishiuji, Takaharu Okada, Masanori Fujii, Hiroshi Kawasaki, Hiroyuki Irie, Miho Shiratori-Hayashi, H. Murota","doi":"10.3389/jcia.2024.12578","DOIUrl":"https://doi.org/10.3389/jcia.2024.12578","url":null,"abstract":"Background: The nature of itch sensation varies depending upon the patient and the disease. However, few studies have focused on verbal expressions describing itch of atopic dermatitis (AD) in quality.Objectives: To investigate itch quality in patients with AD compared with that of urticaria.Methods: We conducted an online questionnaire survey describing itch experiences in June 2021. Participants were Japanese patients who had visited hospitals for their consultations and treatments of AD or urticaria in the last 6 months, and 295 and 290 responses, respectively, to questions using 12 terms describing itch quality were analyzed.Results: The most suitable expression describing intense itch that patients could not help scratching differed between the diseases, where most AD patients selected “muzumuzu” (a mimetic word for creepy–crawly itch) (27%) or “painful itch” (20%), and most urticaria patients selected “muzumuzu” (24%) or “itch like mosquito bites” (22%). The most suitable expressions describing itch that would make patients happiest if improved was “painful itch” (27%) in AD patients, significantly higher than urticaria patients (19%). More AD patients (55%) responded that they sometimes felt itch even after the skin symptoms had subsided than urticaria patients (41%). The most suitable expression of remnant itch selected was “muzumuzu” for AD (58/161 patients, 36%) and urticaria (29/120 patients, 24%).Conclusion: The quality of itch sensations can be classified not only between diseases but also during the clinical course of each disease. Significant expressions that patients with AD use to describe itch sensations could promote more appropriate treatment for itch.","PeriodicalId":508694,"journal":{"name":"Journal of Cutaneous Immunology and Allergy","volume":"265 6‐9","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-04-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140704061","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
A. Yoshizaki, K. Fujii, Atsunori Baba, Hideaki Kawada, T. Kanekura
{"title":"Pyoderma gangrenosum of the mons pubis associated with aplastic anemia","authors":"A. Yoshizaki, K. Fujii, Atsunori Baba, Hideaki Kawada, T. Kanekura","doi":"10.3389/jcia.2024.12845","DOIUrl":"https://doi.org/10.3389/jcia.2024.12845","url":null,"abstract":"","PeriodicalId":508694,"journal":{"name":"Journal of Cutaneous Immunology and Allergy","volume":"36 26","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-04-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140728386","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
S. Morioke, Tatsuki Aikyo, Akio Tanaka, Daiki Matsubara, Ryo Saito, K. Iwamoto, Chisato Yamasaki, Atsushi Kogetsu, Nao Hamakawa, Moeko Isono, B. Yamamoto, Yasushi Matsumura, Toshihiro Takeda, S. Manabe, M. K. Javaid, Joe Barrett, Nathanael Gray, Jane Kaye, Kazuto Kato, M. Hide
Hereditary angioedema (HAE) adversely affects patients’ social and daily life significantly, and the disease burden is high. We recruited study participants from a patient-participatory registry, Rare and Undiagnosed Diseases Study (RUDY) Japan, to better understand the broader effect of HAE on patients’ lives. Thirteen patients with HAE who registered between January 2019 and March 2021 completed an online questionnaire to record details of episodes and the angioedema quality of life (AE-QoL) questionnaire, in which they described the degree to which QoL was impaired. In all, 58 episodes were recorded, and self-reported data were accumulated from 24 returned AE-QoL questionnaires. The episodes peaked between approximately 06:00 and 07:00 h and between approximately 16:00 and 20:00 h. Of the 58 episodes, 38 (65.5%) were treated; 24 (63.2%) were treated at patients’ homes. The drugs used in treatment were hospital-administered human C1-inhibitor preparations for 14 episodes and home-administered icatibant for 26 episodes. Time between episode onset and treatment initiation and between episode onset and full recovery tended to be shorter with icatibant treatment at home. The total AE-QoL score was 37.1 ± 22.9, indicating great impairment of QoL, with particularly high scores in the fatigue/mood and fears/shame domains. Most previous studies of real-world HAE episodes have been retrospective, based on patients’ memories. Collaboration between patients and researchers revealed the location of episodes, the time of day they appeared, treatments for episodes, and the degree to which patients’ QoL was impaired by HAE in this prospective study.
遗传性血管性水肿（HAE）严重影响患者的社交和日常生活，疾病负担沉重。为了更好地了解HAE对患者生活的广泛影响，我们从患者参与的登记处--日本罕见未确诊疾病研究（RUDY）--招募了研究参与者。在2019年1月至2021年3月期间登记的13名HAE患者填写了一份在线问卷，以记录发作的详情和血管性水肿生活质量（AE-QoL）问卷，其中他们描述了生活质量受损的程度。共记录了 58 次发作，并从 24 份收回的 AE-QoL 问卷中积累了自我报告数据。在 58 次发病中，有 38 次（65.5%）接受了治疗；24 次（63.2%）在患者家中接受了治疗。治疗中使用的药物为：14 次在医院使用人 C1 抑制剂制剂，26 次在家中使用 icatibant。在家中使用伊卡替班治疗时，发病与开始治疗之间的时间以及发病与完全康复之间的时间往往较短。AE-QoL总分为37.1±22.9分，表明QoL严重受损，其中疲劳/情绪和恐惧/羞耻领域的得分尤其高。以往对真实世界中 HAE 发作的研究大多是回顾性的，以患者的记忆为基础。在这项前瞻性研究中，患者与研究人员的合作揭示了发作的地点、每天出现的时间、发作时的治疗方法以及 HAE 对患者 QoL 的损害程度。
{"title":"Survey of hereditary angioedema episodes and quality of life impairment through a patient-participatory registry","authors":"S. Morioke, Tatsuki Aikyo, Akio Tanaka, Daiki Matsubara, Ryo Saito, K. Iwamoto, Chisato Yamasaki, Atsushi Kogetsu, Nao Hamakawa, Moeko Isono, B. Yamamoto, Yasushi Matsumura, Toshihiro Takeda, S. Manabe, M. K. Javaid, Joe Barrett, Nathanael Gray, Jane Kaye, Kazuto Kato, M. Hide","doi":"10.3389/jcia.2024.12626","DOIUrl":"https://doi.org/10.3389/jcia.2024.12626","url":null,"abstract":"Hereditary angioedema (HAE) adversely affects patients’ social and daily life significantly, and the disease burden is high. We recruited study participants from a patient-participatory registry, Rare and Undiagnosed Diseases Study (RUDY) Japan, to better understand the broader effect of HAE on patients’ lives. Thirteen patients with HAE who registered between January 2019 and March 2021 completed an online questionnaire to record details of episodes and the angioedema quality of life (AE-QoL) questionnaire, in which they described the degree to which QoL was impaired. In all, 58 episodes were recorded, and self-reported data were accumulated from 24 returned AE-QoL questionnaires. The episodes peaked between approximately 06:00 and 07:00 h and between approximately 16:00 and 20:00 h. Of the 58 episodes, 38 (65.5%) were treated; 24 (63.2%) were treated at patients’ homes. The drugs used in treatment were hospital-administered human C1-inhibitor preparations for 14 episodes and home-administered icatibant for 26 episodes. Time between episode onset and treatment initiation and between episode onset and full recovery tended to be shorter with icatibant treatment at home. The total AE-QoL score was 37.1 ± 22.9, indicating great impairment of QoL, with particularly high scores in the fatigue/mood and fears/shame domains. Most previous studies of real-world HAE episodes have been retrospective, based on patients’ memories. Collaboration between patients and researchers revealed the location of episodes, the time of day they appeared, treatments for episodes, and the degree to which patients’ QoL was impaired by HAE in this prospective study.","PeriodicalId":508694,"journal":{"name":"Journal of Cutaneous Immunology and Allergy","volume":"109 24","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-03-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140370505","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
京公网安备 11010802042870号
京ICP备2023020795号-1
扫码关注我们
求助内容：
应助结果提醒方式：