POEMS syndrome is a rare and disabling autoinflammatory condition characterised by a typical peripheral neuropathy and the presence of a monoclonal plasma cell disorder. The acronym ‘POEMS’ represents the complex and multisystem features of the disease, including polyneuropathy, organomegaly, endocrinopathy, a monoclonal plasma cell disorder and skin disease. The diagnosis of POEMS is a significant challenge because of the heterogeneity of clinical presentations and variation of POEMS features. Patients are often misdiagnosed with another cause of inflammatory neuropathy and receive one or more ineffective immunomodulatory medications, resulting in delayed diagnosis and further clinical deterioration before a diagnosis is made. University College London Hospitals sees one of the largest reported POEMS cohorts in Europe, and runs a multispecialist clinic to assist with diagnosis, treatment and ongoing support. This review draws upon our experience to present the typical features of POEMS syndrome and highlight diagnostic conundrums commonly experienced, supplemented with clinical cases. We provide an investigative guide for clinicians when considering POEMS as the diagnosis, and propose a treatment algorithm that centres on the site and degree of monoclonal cell proliferation.
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Contributors SK and MPTL were responsible for the conception and design of the work. SK collated cases and drafted the paper. All other authors were responsible for interpretation of data, and revisions and edits. All authors agree to be accountable for the work and provide final approval.
Funding SK is funded by an ABN/Guarantors of Brain Clinical Training Research Fellowship [Grant number 541665]. MPTL is supported by the National Institute of Health Research, University College London Hospitals, Biomedical Research Centre.
Competing interests None declared.
Patient consent Obtained.
Ethics approval All patients signed consent forms to allow for their case histories and clinical images to be published.
Provenance and peer review Commissioned; externally peer reviewed. This paper was reviewed by Robert Hadden, London, UK, and Gareth Llewelyn, Cardiff, UK.
Data sharing statement No unpublished data.
Author note VEGF testing is performed currently at the Department of Neuroimmunology at the National Hospital for Neurology and Neurosurgery, London, UK, and has a turnaround time of around 2 weeks.
Correction notice This article has been corrected since it published Online First. A second sentence has been added to the Funding statement.