Introduction: POEMS syndrome is a rare multisystem disorder characterised by polyneuropathy, organomegaly, endocrinopathy, monoclonal plasma-proliferative disorder and skin changes among other features. We describe the course of the endocrine disease in the context of this paraneoplastic syndrome.
Methods: Of the 60 patients with POEMS care in hospital, data are available for 43 patients who attended the Joint POEMS Clinic from 06/1999 to 05/2017. All patients had endocrine screening at every appointment including pituitary, thyroid and bone metabolism. Median and interquartile range and mean±SD are used for quantitative variables and percentage for qualitative variables.
Results: 67% of the patients are male. Age at diagnosis is 50.3[38.263.3] years and the median follow-up is 4.5[1.97.8] years. At diagnosis, 52.5% of the patients presented with endocrinopathy and 47.6% had more than one endocrine disease. From those, 40% had thyroid disease, 27.5% hypogonadism, 5% type 2 diabetes and 4.9% Addison disease.
During surveillance 92.7% of patients developed endocrinopathy. 65.1% of patients had hyperprolactinaemia with a 1.9±0.94 fold increase above the upper limit of the normal, which in 57.1% of the patients it was transient. 62.9% had hypothyroidism (32.6% clinical, 30.3% subclinical), transient in 38.58% of patients. 33.3% presented with high IGF-1 (1.3±0.2 fold increase), and it normalised in 36.4%. Addison disease was diagnosed in 14% of patients with no recovery of the adrenal function. Type 2 diabetes developed in 16.3% of patients and 72.5% had hypogonadism (33.3% secondary, 66.6% primary). Gonadal function recovered in 3 patients. None of them had parathyroid dysfunction.
Conclusion: In our cohort, endocrinopathy in POEMS syndrome was found in 50% at diagnosis and in 90% during follow-up. The multidisciplinary team managing patients with POEMS syndrome should include an endocrinologist. Patients should be systematically assessed for endocrinopathy, which can result in clinically significant symptoms. The most common deficiencies were hypogonadism, and hypothyroidism, both of which were readily remedied with pharmacological intervention. Normalisation of the endocrinopathy was common so on-going treatment thereof should remain under review.
06 Nov 2017 - 08 Nov 2017