Teng Chai Shu, Haydar Ali Tajuddin Amalina, A Wahab Norasyikin, Mustafa Norlaila, Sukor Norlela, Kamaruddin Nor Azmi
The Diabetes and Endocrine Unit, Department of Medicine, National University of Malaysia Medical Centre, Kuala Lumpur, Malaysia.
Int J Endocrinol Metab. 2018 Jun 25;16(3):e65233. doi: 10.5812/ijem.65233. eCollection 2018 Jul.
Ketoconazole has long been the first-line medical therapy for controlling hypercortisolism secondary to either pituitary or adrenal pathology. However, it is largely unavailable in most countries. As a result, we have turned to fluconazole as a viable alternative in view of its favourable safety profile.
A 50-year-old lady developed recurrent Cushing's disease after being in remission following transsphenoidal surgery (TSS) for a left pituitary microadenoma 16 years ago. The repeat MRI showed a right pituitary microadenoma (1.7 mm × 1.3 mm) for which she underwent a second TSS. However, she continued to have persistent hypercortisolism despite repeated MRIs showing absence of tumour recurrence. She refused bilateral adrenalectomy and external radiotherapy. Ketoconazole was commenced at 200 mg twice daily for disease control but this was hindered by intolerable side effects including pruritus and skin exfoliation. In the meantime, she suffered a right hypertensive basal ganglia hemorrhage. Treatment was subsequently switched to cabergoline and the dose titrated to 0.5 mg daily. Fluconazole 400 mg daily was later added to control the persistent disease. Her clinical and biochemical parameters improved markedly three months after the addition of fluconazole. No adverse event was reported. Her disease has remained stable for the last 15 months up until the time of the recent clinic review.
This case demonstrates the long-term efficacy of fluconazole in tandem with cabergoline for the control of recurrent Cushing's disease.
酮康唑长期以来一直是控制继发于垂体或肾上腺病变的皮质醇增多症的一线药物治疗方法。然而,在大多数国家它基本无法获取。因此,鉴于氟康唑良好的安全性,我们已将其作为一种可行的替代药物。
一名50岁女性16年前因左侧垂体微腺瘤接受经蝶窦手术(TSS)后病情缓解,但后来出现复发性库欣病。复查MRI显示右侧垂体微腺瘤(1.7毫米×1.3毫米),为此她接受了第二次TSS。然而,尽管多次MRI显示无肿瘤复发,但她仍持续存在皮质醇增多症。她拒绝双侧肾上腺切除术和外照射放疗。开始使用酮康唑,每日两次,每次200毫克以控制病情,但因出现包括瘙痒和皮肤脱屑等难以耐受的副作用而受阻。与此同时,她发生了右侧高血压性基底节出血。随后治疗改为卡麦角林,剂量滴定至每日0.5毫克。后来添加每日400毫克氟康唑以控制持续性疾病。添加氟康唑三个月后,她的临床和生化参数明显改善。未报告不良事件。直到最近一次门诊复查时,她的病情在过去15个月一直保持稳定。
该病例证明了氟康唑与卡麦角林联合使用对控制复发性库欣病的长期疗效。