Caimari, F;
Keddie, S;
Lunn, MP;
D'Sa, S;
Baldeweg, SE;
(2018)
Prevalence and course of endocrinopathy in POEMS syndrome.
The Journal of Clinical Endocrinology & Metabolism
10.1210/jc.2018-01516.
(In press).
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Lunn_Prevalence and course of endocrinopathy in POEMS syndrome_AAM.pdf - Accepted Version Download (2MB) | Preview |
Abstract
CONTEXT: POEMS syndrome is a rare multisystem disorder characterised by polyneuropathy, organomegaly, endocrinopathy, monoclonal plasma-proliferative disorder and skin changes among other features. OBJECTIVE: To describe the prevalence and course of the endocrine dysfunction in the context of POEMS. DESIGN: Cohort study with systematic review of the endocrinopathy in POEMS. SETTING: 75 patients with POEMS were evaluated by the multidisciplinary team at our tertiary specialist centre. PATIENTS: Endocrine data was available for 59 patients who attended the clinic from 06/1999 to 05/2018. INTERVENTIONS: All patients had regular endocrine screening including testing for diabetes, pituitary and thyroid dysfunction and assessment of bone metabolism. MAIN OUTCOME MEASURE: Prevalence and survival time to develop endocrinopathy in POEMS. RESULTS: Thirty-four (63%) patients presented with an endocrinopathy at point of POEMS diagnosis and 54 (92%) had at least one endocrine abnormality at follow-up. The median follow-up was 4.4[1.5, 7.9] years. The most common endocrine abnormality was hypogonadism in 68%, followed by hyperprolactinaemia (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% in hypogonadism, 42% in hyperprolactinaemia, 34% in hypothyroidism and 38% in high IGF-1 levels. CONCLUSIONS: 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 normalisation of the endocrinopathy can occur so on-going treatment should remain under review.
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