Screening for hereditary angioedema (HAE) at 13 emergency centers in Osaka, Japan

Hirose, Tomoya; Kimbara, Futoshi; Shinozaki, Masaru; Mizushima, Yasuaki; Yamamoto, Hidehiko; Kishi, Masashi; Shiono, Shigeru; Noborio, Mitsuhiro; Fuke, Akihiro; Akimoto, Hiroshi; Kimura, Tadasi; Kaga, Shinichiro; Horiuchi, Takahiko; Shimazu, Takeshi

doi:10.1097/md.0000000000006109

Cited by 9 publications

(10 citation statements)

References 21 publications

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“…In certain countries, particularly Japan, TXA is an important treatment option for many patients with HAE, particularly for LTP. Although C1‐INH has been demonstrated to be safe and effective, self‐administration and LTP are not licensed, and accessibility to treatment is challenging for many patients in Japan . In addition, awareness of HAE among physicians in Japan is low and the limited availability of effective therapies increases the burden of HAE .…”

Section: Discussionmentioning

confidence: 99%

“…In addition, awareness of HAE among physicians in Japan is low and the limited availability of effective therapies increases the burden of HAE . Diagnosis of HAE worldwide can take an average of 8.3 years; this figure is thought to be much longer in Japan . While danazol is available for off‐label use, it has been associated with numerous side effects, including virilization, headaches, depression, and acne.…”

Section: Discussionmentioning

confidence: 99%

“…Although C1-INH has been demonstrated to be safe and | 135 effective, 30,33 self-administration and LTP are not licensed, and accessibility to treatment is challenging for many patients in Japan. 29 In addition, awareness of HAE among physicians in Japan is low and the limited availability of effective therapies increases the burden of HAE. 73 Diagnosis of HAE worldwide can take an average of 8.3 years; this figure is thought to be much longer in Japan.…”

Section: Conclusion Smentioning

confidence: 99%

“…This is a nontypical situation compared with many other regions and is due to limited availability of HAE‐specific therapies. Furthermore, self‐administration of pdC1‐INH is not licensed in Japan, and not all hospitals keep adequate stocks, which can make accessibility difficult for some patients . Importantly, TXA is a more suitable option for patients in whom androgens are contraindicated, for example, women and expectant mothers .…”

Section: Introductionmentioning

confidence: 99%

“…Furthermore, self-administration of pdC1-INH is not licensed in Japan, and not all hospitals keep adequate stocks, which can make accessibility difficult for some patients. 29 Importantly, TXA is a more suitable option for patients in whom androgens are contraindicated, for example, women and expectant mothers. 15 In addition, androgens have been associated with numerous anabolic/androgenic side effects; therefore, treatment requires careful monitoring of patients for the detection of adverse events.…”

mentioning

confidence: 99%

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The use of tranexamic acid for on‐demand and prophylactic treatment of hereditary angioedema—A systematic review

Horiuchi

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Yamashita

et al. 2018

J Cutaneous Imm & Allergy

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Hereditary angioedema (HAE), caused by inherited deficiency of C1 esterase inhibitor (C1‐INH), is characterized by recurring subcutaneous and/or submucosal edema. Although its efficacy remains controversial, tranexamic acid (TXA) is used to treat HAE in some countries. We analyzed TXA as an on‐demand and prophylactic treatment in patients with HAE. Published data were systematically sourced from PubMed and Embase. All retained articles underwent grading/bias assessment using the “SIGN” grading system, and the quality of retained studies was determined following assessment of design and methodology. Of 353 studies identified, 31 were included. On‐demand treatment and prophylactic treatment were assessed in five (N = 103) and 28 studies (N = 231), respectively. The majority of studies (80%) demonstrated that on‐demand TXA was ineffective for skin, abdominal, or laryngeal swellings. In a single randomized controlled trial, the median time to relief of symptoms was 2 and 12 hours for icatibant and TXA, respectively (P < 0.001). For prophylaxis, while ~50% of case series, case reports, and observational studies reported beneficial effects of TXA, newer therapies, for example, icatibant and pdC1‐INH, were more effective. One study found that breakthrough attacks during TXA prophylaxis lasted significantly longer compared with C1‐INH (median time to resolution; 7 vs 3 hours, P = 0.016). Many studies failed to report safety data (16/31, 52%); however, pruritus, vomiting, and diarrhea were noted in some patients. There is no evidence for on‐demand use of TXA in HAE and limited evidence for prophylaxis. While TXA may be more beneficial than no treatment, newer, more effective therapies should be used when available.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%