Department of Endocrinology, University College London Hospitals NHS Foundation Trust, London United Kingdom.
Medical Research Council Centre for Neuromuscular Disease, National Hospital of Neurology and Neurosurgery, London, United Kingdom.
J Clin Endocrinol Metab. 2019 Jun 1;104(6):2140-2146. doi: 10.1210/jc.2018-01516.
POEMS syndrome is a rare multisystem disorder characterized by polyneuropathy, organomegaly, endocrinopathy, monoclonal plasma-proliferative disorder, and skin changes, among other features.
To describe the prevalence and course of endocrine dysfunction in POEMS.
Cohort study with systematic review of the endocrinopathy in POEMS.
Seventy-five patients with POEMS were evaluated by the multidisciplinary team at our tertiary specialist center.
Endocrine data were available for 59 patients who attended the clinic from June 1999 to May 2018.
All patients had regular endocrine screening, including testing for diabetes, pituitary and thyroid dysfunction and assessment of bone metabolism.
Prevalence and survival time to develop endocrinopathy in POEMS.
Thirty-four (63%) patients presented with an endocrinopathy at POEMS diagnosis and 54 (92%) had at least one endocrine abnormality at follow-up. The median follow-up was 4.4 (interquartile range, 1.5, 7.9) years. The most common endocrine abnormality was hypogonadism in 68%, followed by hyperprolactinemia (56%), hypothyroidism (54%), abnormal glucose metabolism (24%), adrenal insufficiency (17%), and high IGF-1 levels (15%). Spontaneous resolution of endocrine abnormalities at the end of follow-up was observed: 14% of patients with hypogonadism; 42%, hyperprolactinemia; 34%, hypothyroidism; and 38%, high IGF-1 levels.
Endocrinopathy was found in 63% of patients at diagnosis and in 92% of patients during follow-up in our cohort. Therefore, patients with POEMS should be systematically assessed for endocrinopathy. The most common deficiencies were hypogonadism and hypothyroidism; however, but endocrinopathy can normalize, so ongoing treatment should remain under review.
POEMS 综合征是一种罕见的多系统疾病，其特征为多发性神经病、器官肿大、内分泌病、单克隆浆细胞增殖性疾病和皮肤改变等。
描述 POEMS 中内分泌功能障碍的发生率和病程。
对我们的三级专科中心多学科团队评估的 75 例 POEMS 患者进行队列研究并进行 POEMS 内分泌病的系统评价。
75 例 POEMS 患者在我们的三级专科中心接受评估。
内分泌数据可用于 1999 年 6 月至 2018 年 5 月期间在诊所就诊的 59 例患者。
所有患者均进行常规内分泌筛查，包括糖尿病、垂体和甲状腺功能障碍的检测以及骨代谢评估。
POEMS 中内分泌疾病的发生率和发病时间。
34 例（63%）患者在 POEMS 诊断时出现内分泌疾病，54 例（92%）在随访时至少存在 1 项内分泌异常。中位随访时间为 4.4 年（四分位距，1.5~7.9）。最常见的内分泌异常是性腺功能减退症，占 68%，其次是高催乳素血症（56%）、甲状腺功能减退症（54%）、葡萄糖代谢异常（24%）、肾上腺皮质功能不全（17%）和 IGF-1 水平升高（15%）。在随访结束时观察到内分泌异常的自发缓解：14%的性腺功能减退症患者；42%的高催乳素血症患者；34%的甲状腺功能减退症患者；38%的 IGF-1 水平升高患者。
在我们的队列中，63%的患者在诊断时和 92%的患者在随访期间存在内分泌疾病。因此，POEMS 患者应系统评估内分泌疾病。最常见的缺陷是性腺功能减退症和甲状腺功能减退症；然而，内分泌疾病可以正常化，因此应持续审查正在进行的治疗。
