Treatment of hereditary angioneurotic oedema (HANE) with tibolone

Abstract

Tibolone may represent an alternative to danazol administration for the prophylaxis of HANE in women.

WAO Guideline for the Management of Hereditary Angioedema

“…Results of search 8: Munch and Weeke, [3] Lewis, [4] Gluszko et al, [5] Cicardi et al, [6] Waytes et al, [7] Cicardi et al, [8] Goring et al, [9] Kunschak et al, [10] Farkas et al, [11] Bork and Barnstedt, [12] Gompels et al, [13] Weiler et al, [14] Szeplaki et al, [15] van Doorn et al, [16] Bas et al, [17] Bork et al, [18] Ott et al, [19] Schneider et al, [20] Varga et al, [21] Visy et al, [22] Birjmohun et al, [23] Cedzynski et al, [24] Szegedi et al, [25] Craig et al, [26] Kreuz et al, [27] Bas et al, [28] Bernstein et al, [29] Cicardi et al, [30] Cicardi et al, [31] Craig et al, [32] Levy et al, [33] Zuraw et al, [34] Zuraw et al [35]. …”

“…Results of search 13: Cicardi et al, [8] Farkas et al, [11] Szeplaki et al, [15] Ott et al, [19] Birjmohun et al, [23] and Szegedi et al [25]. …”

International consensus and practical guidelines on the gynecologic and obstetric management of female patients with hereditary angioedema caused by C1 inhibitor deficiency

“…It has been used in 8 premenopausal and postmenopausal women with HAE-C1-INH subtype I, with good clinical results. 153 However, there is no information on the contraceptive effects of this compound in premenopausal women.…”

“…167,168 Tibolone, a normethyltestosterone derivative used in symptomatic postmenopausal women, can also be used but should be given in place of rather than in combination with danazol because of a risk of greater side-effects because of their common properties. 153 Nonhormonal alternatives, such as selective serotonin reuptake inhibitors, serotonin-norepinephrine reuptake inhibitors, and clonidine can be used for resistant hot flushes and insomnia. 169 As for selective estrogen receptor modulators, there are no reports about the use of raloxifene in patients with HAE-C1-INH, but a report on tamoxifen as an adjuvant breast cancer therapy described a worsening of HAE-C1-INH sympotms.…”

Clinical Immunology Review Series: An approach to the patient with angio-oedema

“…For patients who experience frequent or life‐threatening attacks, oral prophylaxis is recommended. Methyl‐testosterone or the 17 α‐alkylated attenuated androgens, such as danazol [108], stanozolol [109], oxandrolone [110] and tibolone [111], reduce frequency of attacks for most patients. They probably work by increasing production of C1 INH by hepatocytes.…”