Clinical characteristics, risk factors, and outcomes of POEMS syndrome: A longitudinal cohort study.

Adult Aged Aged, 80 and over Cohort Studies Female Follow-Up Studies Humans Longitudinal Studies Male Middle Aged POEMS Syndrome / diagnosis Retrospective Studies Risk Factors Treatment Outcome

Journal

Neurology
ISSN: 1526-632X
Titre abrégé: Neurology
Pays: United States
ID NLM: 0401060

Informations de publication

Date de publication:
21 07 2020
Historique:
received: 17 07 2019
accepted: 27 12 2019
pubmed: 2 7 2020
medline: 2 10 2020
entrez: 2 7 2020
Statut: ppublish

Résumé

POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin lesions) is a paraneoplastic disorder resulting in severe neurologic disability. Understanding the clinical, laboratory, neurophysiologic, and histopathologic features as well as treatment responses of POEMS will assist in more accurate and timely diagnosis, risk stratification, and effective management. This was a retrospective longitudinal cohort study from 1998 to March 2019, with 7,184 person-months of follow-up time. Hospital databases were used to collate presenting features, investigations, therapies, and response. One hundred patients were included with a median follow-up time of 59 months (range, 1-252). Mean symptom onset to diagnosis was 15 months (range, 1-77), with 54% of patients initially misdiagnosed with chronic inflammatory demyelinating polyneuropathy. Median number of multisystem features at diagnosis was 7. Ninety-six (96%) presented with neuropathy, which was length-dependent in 93 (93%) and painful in 75 (75%). At diagnosis, 35% of patients were wheelchair or bedbound, with median Overall Neuropathy Limitation Score of 6, improving to 3 following treatment ( POEMS syndrome is a rare multisystem condition with delayed diagnosis and poor neurologic function at presentation. Therapy has favorable outcomes. Patients at high risk of death or progression can be identified, which may allow for more active monitoring and influence management.

Identifiants

DOI: 10.1212/WNL.0000000000009940 PMID: 32606227
pubmed: 32606227
pii: WNL.0000000000009940
doi: 10.1212/WNL.0000000000009940
doi:

Types de publication

Journal Article Research Support, Non-U.S. Gov't

Langues

eng

Sous-ensembles de citation

IM

Pagination

e268-e279

Subventions

Organisme : Medical Research Council
ID : MR/P008399/1
Pays : United Kingdom
Organisme : Department of Health
Pays : United Kingdom

Informations de copyright

© 2020 American Academy of Neurology.

Auteurs

Stephen Keddie (S)

From the MRC Centre for Neuromuscular Disease, Neuroimmunology and CSF Laboratory (S.K., O.J.Z., H.M., A.C., M.M.R., M.P.L.), Institute of Neurology, Cancer Division (D.F., J.B., A.C., K.Y., S.D.), Department of Endocrinology (F.C., S.E.B., M.P.L.), and Division of Neuropathology, National Hospital for Neurology and Neurosurgery (Z.J., S.B.), University College London NHS Foundation Trust (S.K.), National Hospital for Neurology and Neurosurgery; Department of Neuromuscular Diseases (S.K., O.J.Z., H.M., A.C., M.M.R., M.P.L.), UCL Queen Square Institute of Neurology; and Department of Clinical Neurosciences (J.F., S.R.), John Radcliffe Hospital, Oxford, UK.
ORCID: 0000-0002-2102-6053

David Foldes (D)

From the MRC Centre for Neuromuscular Disease, Neuroimmunology and CSF Laboratory (S.K., O.J.Z., H.M., A.C., M.M.R., M.P.L.), Institute of Neurology, Cancer Division (D.F., J.B., A.C., K.Y., S.D.), Department of Endocrinology (F.C., S.E.B., M.P.L.), and Division of Neuropathology, National Hospital for Neurology and Neurosurgery (Z.J., S.B.), University College London NHS Foundation Trust (S.K.), National Hospital for Neurology and Neurosurgery; Department of Neuromuscular Diseases (S.K., O.J.Z., H.M., A.C., M.M.R., M.P.L.), UCL Queen Square Institute of Neurology; and Department of Clinical Neurosciences (J.F., S.R.), John Radcliffe Hospital, Oxford, UK.

Francisca Caimari (F)

From the MRC Centre for Neuromuscular Disease, Neuroimmunology and CSF Laboratory (S.K., O.J.Z., H.M., A.C., M.M.R., M.P.L.), Institute of Neurology, Cancer Division (D.F., J.B., A.C., K.Y., S.D.), Department of Endocrinology (F.C., S.E.B., M.P.L.), and Division of Neuropathology, National Hospital for Neurology and Neurosurgery (Z.J., S.B.), University College London NHS Foundation Trust (S.K.), National Hospital for Neurology and Neurosurgery; Department of Neuromuscular Diseases (S.K., O.J.Z., H.M., A.C., M.M.R., M.P.L.), UCL Queen Square Institute of Neurology; and Department of Clinical Neurosciences (J.F., S.R.), John Radcliffe Hospital, Oxford, UK.

Stephanie E Baldeweg (SE)

From the MRC Centre for Neuromuscular Disease, Neuroimmunology and CSF Laboratory (S.K., O.J.Z., H.M., A.C., M.M.R., M.P.L.), Institute of Neurology, Cancer Division (D.F., J.B., A.C., K.Y., S.D.), Department of Endocrinology (F.C., S.E.B., M.P.L.), and Division of Neuropathology, National Hospital for Neurology and Neurosurgery (Z.J., S.B.), University College London NHS Foundation Trust (S.K.), National Hospital for Neurology and Neurosurgery; Department of Neuromuscular Diseases (S.K., O.J.Z., H.M., A.C., M.M.R., M.P.L.), UCL Queen Square Institute of Neurology; and Department of Clinical Neurosciences (J.F., S.R.), John Radcliffe Hospital, Oxford, UK.

Joshua Bomsztyk (J)

From the MRC Centre for Neuromuscular Disease, Neuroimmunology and CSF Laboratory (S.K., O.J.Z., H.M., A.C., M.M.R., M.P.L.), Institute of Neurology, Cancer Division (D.F., J.B., A.C., K.Y., S.D.), Department of Endocrinology (F.C., S.E.B., M.P.L.), and Division of Neuropathology, National Hospital for Neurology and Neurosurgery (Z.J., S.B.), University College London NHS Foundation Trust (S.K.), National Hospital for Neurology and Neurosurgery; Department of Neuromuscular Diseases (S.K., O.J.Z., H.M., A.C., M.M.R., M.P.L.), UCL Queen Square Institute of Neurology; and Department of Clinical Neurosciences (J.F., S.R.), John Radcliffe Hospital, Oxford, UK.

Oliver J Ziff (OJ)

From the MRC Centre for Neuromuscular Disease, Neuroimmunology and CSF Laboratory (S.K., O.J.Z., H.M., A.C., M.M.R., M.P.L.), Institute of Neurology, Cancer Division (D.F., J.B., A.C., K.Y., S.D.), Department of Endocrinology (F.C., S.E.B., M.P.L.), and Division of Neuropathology, National Hospital for Neurology and Neurosurgery (Z.J., S.B.), University College London NHS Foundation Trust (S.K.), National Hospital for Neurology and Neurosurgery; Department of Neuromuscular Diseases (S.K., O.J.Z., H.M., A.C., M.M.R., M.P.L.), UCL Queen Square Institute of Neurology; and Department of Clinical Neurosciences (J.F., S.R.), John Radcliffe Hospital, Oxford, UK.

Janev Fehmi (J)

From the MRC Centre for Neuromuscular Disease, Neuroimmunology and CSF Laboratory (S.K., O.J.Z., H.M., A.C., M.M.R., M.P.L.), Institute of Neurology, Cancer Division (D.F., J.B., A.C., K.Y., S.D.), Department of Endocrinology (F.C., S.E.B., M.P.L.), and Division of Neuropathology, National Hospital for Neurology and Neurosurgery (Z.J., S.B.), University College London NHS Foundation Trust (S.K.), National Hospital for Neurology and Neurosurgery; Department of Neuromuscular Diseases (S.K., O.J.Z., H.M., A.C., M.M.R., M.P.L.), UCL Queen Square Institute of Neurology; and Department of Clinical Neurosciences (J.F., S.R.), John Radcliffe Hospital, Oxford, UK.

Aviva Cerner (A)

From the MRC Centre for Neuromuscular Disease, Neuroimmunology and CSF Laboratory (S.K., O.J.Z., H.M., A.C., M.M.R., M.P.L.), Institute of Neurology, Cancer Division (D.F., J.B., A.C., K.Y., S.D.), Department of Endocrinology (F.C., S.E.B., M.P.L.), and Division of Neuropathology, National Hospital for Neurology and Neurosurgery (Z.J., S.B.), University College London NHS Foundation Trust (S.K.), National Hospital for Neurology and Neurosurgery; Department of Neuromuscular Diseases (S.K., O.J.Z., H.M., A.C., M.M.R., M.P.L.), UCL Queen Square Institute of Neurology; and Department of Clinical Neurosciences (J.F., S.R.), John Radcliffe Hospital, Oxford, UK.

Zane Jaunmuktane (Z)

From the MRC Centre for Neuromuscular Disease, Neuroimmunology and CSF Laboratory (S.K., O.J.Z., H.M., A.C., M.M.R., M.P.L.), Institute of Neurology, Cancer Division (D.F., J.B., A.C., K.Y., S.D.), Department of Endocrinology (F.C., S.E.B., M.P.L.), and Division of Neuropathology, National Hospital for Neurology and Neurosurgery (Z.J., S.B.), University College London NHS Foundation Trust (S.K.), National Hospital for Neurology and Neurosurgery; Department of Neuromuscular Diseases (S.K., O.J.Z., H.M., A.C., M.M.R., M.P.L.), UCL Queen Square Institute of Neurology; and Department of Clinical Neurosciences (J.F., S.R.), John Radcliffe Hospital, Oxford, UK.

Sebastian Brandner (S)

From the MRC Centre for Neuromuscular Disease, Neuroimmunology and CSF Laboratory (S.K., O.J.Z., H.M., A.C., M.M.R., M.P.L.), Institute of Neurology, Cancer Division (D.F., J.B., A.C., K.Y., S.D.), Department of Endocrinology (F.C., S.E.B., M.P.L.), and Division of Neuropathology, National Hospital for Neurology and Neurosurgery (Z.J., S.B.), University College London NHS Foundation Trust (S.K.), National Hospital for Neurology and Neurosurgery; Department of Neuromuscular Diseases (S.K., O.J.Z., H.M., A.C., M.M.R., M.P.L.), UCL Queen Square Institute of Neurology; and Department of Clinical Neurosciences (J.F., S.R.), John Radcliffe Hospital, Oxford, UK.

Kwee Yong (K)

From the MRC Centre for Neuromuscular Disease, Neuroimmunology and CSF Laboratory (S.K., O.J.Z., H.M., A.C., M.M.R., M.P.L.), Institute of Neurology, Cancer Division (D.F., J.B., A.C., K.Y., S.D.), Department of Endocrinology (F.C., S.E.B., M.P.L.), and Division of Neuropathology, National Hospital for Neurology and Neurosurgery (Z.J., S.B.), University College London NHS Foundation Trust (S.K.), National Hospital for Neurology and Neurosurgery; Department of Neuromuscular Diseases (S.K., O.J.Z., H.M., A.C., M.M.R., M.P.L.), UCL Queen Square Institute of Neurology; and Department of Clinical Neurosciences (J.F., S.R.), John Radcliffe Hospital, Oxford, UK.

Hadi Manji (H)

From the MRC Centre for Neuromuscular Disease, Neuroimmunology and CSF Laboratory (S.K., O.J.Z., H.M., A.C., M.M.R., M.P.L.), Institute of Neurology, Cancer Division (D.F., J.B., A.C., K.Y., S.D.), Department of Endocrinology (F.C., S.E.B., M.P.L.), and Division of Neuropathology, National Hospital for Neurology and Neurosurgery (Z.J., S.B.), University College London NHS Foundation Trust (S.K.), National Hospital for Neurology and Neurosurgery; Department of Neuromuscular Diseases (S.K., O.J.Z., H.M., A.C., M.M.R., M.P.L.), UCL Queen Square Institute of Neurology; and Department of Clinical Neurosciences (J.F., S.R.), John Radcliffe Hospital, Oxford, UK.

Aisling Carr (A)

From the MRC Centre for Neuromuscular Disease, Neuroimmunology and CSF Laboratory (S.K., O.J.Z., H.M., A.C., M.M.R., M.P.L.), Institute of Neurology, Cancer Division (D.F., J.B., A.C., K.Y., S.D.), Department of Endocrinology (F.C., S.E.B., M.P.L.), and Division of Neuropathology, National Hospital for Neurology and Neurosurgery (Z.J., S.B.), University College London NHS Foundation Trust (S.K.), National Hospital for Neurology and Neurosurgery; Department of Neuromuscular Diseases (S.K., O.J.Z., H.M., A.C., M.M.R., M.P.L.), UCL Queen Square Institute of Neurology; and Department of Clinical Neurosciences (J.F., S.R.), John Radcliffe Hospital, Oxford, UK.

Simon Rinaldi (S)

From the MRC Centre for Neuromuscular Disease, Neuroimmunology and CSF Laboratory (S.K., O.J.Z., H.M., A.C., M.M.R., M.P.L.), Institute of Neurology, Cancer Division (D.F., J.B., A.C., K.Y., S.D.), Department of Endocrinology (F.C., S.E.B., M.P.L.), and Division of Neuropathology, National Hospital for Neurology and Neurosurgery (Z.J., S.B.), University College London NHS Foundation Trust (S.K.), National Hospital for Neurology and Neurosurgery; Department of Neuromuscular Diseases (S.K., O.J.Z., H.M., A.C., M.M.R., M.P.L.), UCL Queen Square Institute of Neurology; and Department of Clinical Neurosciences (J.F., S.R.), John Radcliffe Hospital, Oxford, UK.

Mary M Reilly (MM)

From the MRC Centre for Neuromuscular Disease, Neuroimmunology and CSF Laboratory (S.K., O.J.Z., H.M., A.C., M.M.R., M.P.L.), Institute of Neurology, Cancer Division (D.F., J.B., A.C., K.Y., S.D.), Department of Endocrinology (F.C., S.E.B., M.P.L.), and Division of Neuropathology, National Hospital for Neurology and Neurosurgery (Z.J., S.B.), University College London NHS Foundation Trust (S.K.), National Hospital for Neurology and Neurosurgery; Department of Neuromuscular Diseases (S.K., O.J.Z., H.M., A.C., M.M.R., M.P.L.), UCL Queen Square Institute of Neurology; and Department of Clinical Neurosciences (J.F., S.R.), John Radcliffe Hospital, Oxford, UK.

Shirley D'Sa (S)

From the MRC Centre for Neuromuscular Disease, Neuroimmunology and CSF Laboratory (S.K., O.J.Z., H.M., A.C., M.M.R., M.P.L.), Institute of Neurology, Cancer Division (D.F., J.B., A.C., K.Y., S.D.), Department of Endocrinology (F.C., S.E.B., M.P.L.), and Division of Neuropathology, National Hospital for Neurology and Neurosurgery (Z.J., S.B.), University College London NHS Foundation Trust (S.K.), National Hospital for Neurology and Neurosurgery; Department of Neuromuscular Diseases (S.K., O.J.Z., H.M., A.C., M.M.R., M.P.L.), UCL Queen Square Institute of Neurology; and Department of Clinical Neurosciences (J.F., S.R.), John Radcliffe Hospital, Oxford, UK.

Michael P Lunn (MP)

From the MRC Centre for Neuromuscular Disease, Neuroimmunology and CSF Laboratory (S.K., O.J.Z., H.M., A.C., M.M.R., M.P.L.), Institute of Neurology, Cancer Division (D.F., J.B., A.C., K.Y., S.D.), Department of Endocrinology (F.C., S.E.B., M.P.L.), and Division of Neuropathology, National Hospital for Neurology and Neurosurgery (Z.J., S.B.), University College London NHS Foundation Trust (S.K.), National Hospital for Neurology and Neurosurgery; Department of Neuromuscular Diseases (S.K., O.J.Z., H.M., A.C., M.M.R., M.P.L.), UCL Queen Square Institute of Neurology; and Department of Clinical Neurosciences (J.F., S.R.), John Radcliffe Hospital, Oxford, UK. michaellunn@nhs.net.
ORCID: 0000-0003-3174-6027

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