To identify the presence, distribution, severity and frequency of autonomic dysfunction in polyneuropathy, organomegaly, endocrinopathy, monoclonal plasma cell-proliferative disorder, skin changes (POEMS) syndrome.

Autonomic dysfunction in POEMS syndrome has only been reported anecdotally in literature with other small studies reporting a lack of involvement that may be due to small numbers of patients. The real frequency, severity, distribution and temporal profile of autonomic involvement in POEMS remain unknown.

We retrospectively reviewed the medical charts of 138 POEMS patients seen at Mayo Clinic between 1979 and 2007 for autonomic symptoms and autonomic testing including autonomic reflex screen which evaluates the postganglionic sudomotor, cardiovagal and adrenergic function and thermoregulatory sweat test. We then used the composite Autonomic Severity Scores (CASS) to determine the distribution and severity of autonomic dysfunction.

Eighty five patients had autonomic complaints including erectile dysfunction, early satiety, constipation, diarrhea, urinary incontinence and orthostatic hypotension. Forty had an autonomic reflex screen and 6 had a thermoregulatory sweat test. Postganglionic sudomotor dysfunction was found in 16, cardiovagal impairment in 13 and adrenergic failure in 5. Distal anhidrosis was found in 4/6 patients who underwent a thermoregulatory sweat test. CASS scores ranged from 0 to 6 with a mean of 1.7. Interestingly, 7 patients underwent serial autonomic reflex screens and 6 of those showed some progression of their CASS over time denoting worsening autonomic dysfunction.

Our retrospective analysis suggests that autonomic dysfunction in POEMS is present and usually mild. Thus, patients should be questioned about autonomic symptoms, tested and treated accordingly which may positively affect their outcome. Prospective studies of the autonomic involvement in POEMS are required to further establish its incidence and relationship with progression of the disease.