Endocrinology in Charlottenburg, Berlin, Germany.
Department of Endocrinology and Department of Medical and Health Sciences, Linköping University, Linköping, Sweden.
Clin Endocrinol (Oxf). 2018 Jul;89(1):30-35. doi: 10.1111/cen.13609. Epub 2018 Apr 22.
Mortality from primary and secondary adrenal insufficiency (AI; PAI and SAI, respectively) is 2-3-fold higher than in the general population. Mortality relates to cardiovascular disease, acute adrenal crisis (AC), cancer and infections; however, there has been little further characterization of patients who have died.
DESIGN/METHODS: We analysed real-world data from 2034 patients (801 PAI, 1233 SAI) in the European Adrenal Insufficiency Registry (EU-AIR; NCT01661387). Baseline clinical and biochemical data of patients who subsequently died were compared with those who remained alive.
From August 2012 to June 2017, 26 deaths occurred (8 PAI, 18 SAI) from cardiovascular disease (n = 9), infection (n = 4), suicide (n = 2), drug-induced hepatitis (n = 2), and renal failure, brain tumour, cachexia and AC (each n = 1); cause of death was unclear in 5 patients. Patients who died were significantly older at baseline than alive patients. Causes of AI were representative of patients with SAI; however, 3-quarters of deceased patients with PAI had undergone bilateral adrenalectomy (3 with uncontrolled Cushing's disease, 3 with metastatic renal cell cancer). There were no significant differences in body mass index, blood pressure, low-density lipoprotein cholesterol, total cholesterol or electrolytes between deceased and alive patients. Deceased patients with SAI were more frequently male individuals, were receiving higher daily doses of hydrocortisone (24.0 ± 7.6 vs 19.3 ± 5.7 mg, P = .0016) and experienced more frequent ACs (11.1 vs 2.49/100 patient-years, P = .0389) than alive patients.
This is the first study to provide detailed characteristics of deceased patients with AI. Older, male patients with SAI and frequent AC had a high mortality risk.
原发性和继发性肾上腺功能不全（PAI 和 SAI）的死亡率比普通人群高 2-3 倍。死亡率与心血管疾病、急性肾上腺危象（AC）、癌症和感染有关；然而，对于已经死亡的患者，我们对其特征的了解甚少。
方法/设计：我们分析了欧洲肾上腺功能不全登记处（EU-AIR；NCT01661387）2034 例患者（801 例 PAI，1233 例 SAI）的真实世界数据。随后死亡的患者的基线临床和生化数据与仍存活的患者进行了比较。
从 2012 年 8 月至 2017 年 6 月，26 例死亡（8 例 PAI，18 例 SAI），原因包括心血管疾病（9 例）、感染（4 例）、自杀（2 例）、药物性肝炎（2 例）、肾衰竭、脑肿瘤、恶病质和 AC（各 1 例）；5 例患者的死因不明。与存活患者相比，死亡患者的基线年龄明显更大。AI 的病因与 SAI 患者相似；然而，75%的 PAI 死亡患者曾接受过双侧肾上腺切除术（3 例患有无法控制的库欣病，3 例患有转移性肾细胞癌）。死亡和存活患者的体重指数、血压、低密度脂蛋白胆固醇、总胆固醇或电解质均无显著差异。患有 SAI 的死亡男性患者更为常见，接受的氢化可的松日剂量更高（24.0±7.6 比 19.3±5.7mg，P=0.0016），且经历的 AC 更为频繁（11.1 比 2.49/100 患者年，P=0.0389）。
这是第一项详细描述 AI 死亡患者特征的研究。年龄较大、男性、患有 SAI 和频繁发生 AC 的患者具有较高的死亡风险。
