Wan Muhammad Hatta S F, Kandaswamy L, Gherman-Ciolac C, Mann J, Buch H N
New Cross Hospital, Wolverhampton, UK.
Faculty of Medicine, Universiti Teknologi MARA, Sungai Buloh Campus, 47000 Sungai Buloh, Selangor, Malaysia.
Endocrinol Diabetes Metab Case Rep. 2018 Jul 26;2018. doi: 10.1530/EDM-18-0074. eCollection 2018.
Myopathy is a well-known complication of hypercortisolism and commonly involves proximal lower-limb girdle. We report a rare case of Cushing's syndrome in a 60-year-old female presenting with significant respiratory muscle weakness and respiratory failure. She had history of rheumatoid arthritis, primary biliary cirrhosis and primary hypothyroidism and presented with weight gain and increasing shortness of breath. Investigations confirmed a restrictive defect with impaired gas transfer but with no significant parenchymatous pulmonary disease. Respiratory muscle test confirmed weakness of respiratory muscles and diaphragm. Biochemical and radiological investigations confirmed hypercortisolaemia secondary to a left adrenal tumour. Following adrenalectomy her respiratory symptoms improved along with an objective improvement in the respiratory muscle strength, diaphragmatic movement and pulmonary function test.
Cushing's syndrome can present in many ways, a high index of suspicion is required for its diagnosis, as often patients present with only few of the pathognomonic symptoms and signs of the syndrome.Proximal lower-limb girdle myopathy is common in Cushing's syndrome. Less often long-term exposure of excess glucocorticoid production can also affect other muscles including respiratory muscle and the diaphragm leading to progressive shortness of breath and even acute respiratory failure.Treatment of Cushing's myopathy involves treating the underlying cause that is hypercortisolism. Various medications have been suggested to hinder the development of GC-induced myopathy, but their effects are poorly analysed.
肌病是皮质醇增多症的一种常见并发症,通常累及近端下肢带。我们报告一例60岁女性库欣综合征罕见病例,该患者出现明显的呼吸肌无力和呼吸衰竭。她有类风湿关节炎、原发性胆汁性肝硬化和原发性甲状腺功能减退病史,表现为体重增加和气短加重。检查证实存在限制性通气障碍伴气体交换受损,但无明显实质性肺部疾病。呼吸肌测试证实呼吸肌和膈肌无力。生化和影像学检查证实继发于左肾上腺肿瘤的高皮质醇血症。肾上腺切除术后,她的呼吸症状改善,呼吸肌力量、膈肌运动和肺功能测试也有客观改善。
库欣综合征可表现为多种形式,其诊断需要高度怀疑,因为患者通常仅表现出该综合征的少数典型症状和体征。近端下肢带肌病在库欣综合征中很常见。长期过量产生糖皮质激素较少情况下也会影响包括呼吸肌和膈肌在内的其他肌肉,导致进行性气短甚至急性呼吸衰竭。库欣肌病的治疗包括治疗根本病因,即高皮质醇血症。已提出各种药物来阻碍糖皮质激素诱导的肌病发展,但对其效果分析不足。