Prevalence and Course of Endocrinopathy in POEMS Syndrome

Context: POEMS syndrome is a rare multisystem disorder characterized by polyneuropathy, organomegaly, endocrinopathy, monoclonal plasma-proliferative disorder, and skin changes, among other features. Objective: To describe the prevalence and course of endocrine dysfunction in POEMS. Design: Cohort study with systematic review of the endocrinopathy in POEMS. Setting: Seventy-five patients with POEMS were evaluated by the multidisciplinary team at our tertiary specialist center. Patients: Endocrine data were available for 59 patients who attended the clinic from June 1999 to May 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 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. 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, but endocrinopathy can normalize, so ongoing treatment should remain under review.