Prevalence and Course of Endocrinopathy in POEMS Syndrome

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.