Livadas S, Androulakis I, Angelopoulos N, Lytras A, Papagiannopoulos F, Kassi G
Endocrine Unit, Metropolitan Hospital, Athens, Greece.
Novo-Nordisk, Athens, Greece.
Endocrinol Diabetes Metab Case Rep. 2020 Jun 4;2020. doi: 10.1530/EDM-19-0150.
HAIR-AN syndrome, the coexistence of Hirsutism, Insulin Resistance (IR) and Acanthosis Nigricans, constitutes a rare nosologic entity. It is characterized from clinical and biochemical hyperandrogenism accompanied with severe insulin resistance, chronic anovulation and metabolic abnormalities. Literally, HAIR-AN represents an extreme case of polycystic ovary syndrome (PCOS). In everyday practice, the management of HAIR-AN constitutes a therapeutic challenge with the available pharmaceutical agents. Specifically, the degree of IR cannot be significantly ameliorated with metformin administration, whereas oral contraceptives chronic administration is associated with worsening of metabolic profile. Liraglutide and exenatide, in combination with metformin, have been introduced in the management of significantly obese women with PCOS with satisfactory results. Based on this notion, we prescribed liraglutide in five women with HAIR-AN. In all participants a significant improvement regarding the degree of IR, fat depositions, androgen levels and the pattern of menstrual cycle was observed, with minimal weight loss. Furthermore, one woman became pregnant during liraglutide treatment giving birth to a healthy child. Accordingly, we conclude that liraglutide constitutes an effective alternative in the management of women with HAIR-AN.
HAIR-AN management is challenging and classic therapeutic regimens are ineffective. Literally HAIR-AN syndrome, the coexistence of Hirsutism, Insulin Resistance and Acanthosis Nigricans, represents an extreme case of polycystic ovary syndrome. In cases of HAIR-AN, liraglutide constitutes an effective and safe choice.
HAIR-AN综合征是多毛症、胰岛素抵抗(IR)和黑棘皮病同时存在的一种罕见病症。其特征为临床和生化方面的高雄激素血症,伴有严重的胰岛素抵抗、慢性无排卵和代谢异常。从字面上看,HAIR-AN代表多囊卵巢综合征(PCOS)的一种极端情况。在日常临床实践中,使用现有的药物治疗HAIR-AN是一项治疗挑战。具体而言,二甲双胍治疗并不能显著改善IR程度,而长期口服避孕药会导致代谢状况恶化。利拉鲁肽和艾塞那肽与二甲双胍联合应用,已被用于治疗重度肥胖的PCOS女性患者,并取得了满意的效果。基于这一理念,我们对5例HAIR-AN女性患者使用了利拉鲁肽。所有参与者的IR程度、脂肪沉积、雄激素水平和月经周期模式均有显著改善,体重减轻极少。此外,1例女性在利拉鲁肽治疗期间怀孕并诞下一名健康婴儿。因此,我们得出结论,利拉鲁肽是治疗HAIR-AN女性患者的一种有效替代药物。
HAIR-AN的治疗具有挑战性,经典治疗方案无效。从字面上看,HAIR-AN综合征即多毛症、胰岛素抵抗和黑棘皮病同时存在,是多囊卵巢综合征的一种极端情况。对于HAIR-AN病例,利拉鲁肽是一种有效且安全的选择。