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Case Reports in Medicine
Volume 2017, Article ID 1276759, 6 pages
https://doi.org/10.1155/2017/1276759
Case Report

Polyneuropathy as Novel Initial Manifestation in a Case of “Nonsecretory” POEMS Syndrome with Sjögren’s Syndrome

1Division of Rheumatology, Huashan Hospital, Shanghai, China
2Institute of Rheumatology, Immunology and Allergy, Fudan University, Shanghai 200040, China
3Department of Hematology, Huashan Hospital, Fudan University, Shanghai 200040, China
4Nursing Department, Huashan Hospital, Fudan University, Shanghai 200040, China

Correspondence should be addressed to Weiguo Wan; moc.anis@9691nawgw and Jun Liu; moc.nuyila@nujuilnahsauh

Received 9 July 2016; Revised 21 September 2016; Accepted 12 October 2016; Published 4 January 2017

Academic Editor: Arduino A. Mangoni

Copyright © 2017 Minrui Liang et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes) is a paraneoplastic syndrome driven by plasma cell dyscrasias. We report a patient with novel initial manifestation of polyneuropathy, which was considered due to Sjögren’s syndrome but with poor response to methylprednisolone (120 mg/d) and intravenous immunoglobulin (IVIg). Further investigation by imaging tests and following biopsy eventually confirmed the diagnosis of POEMS syndrome secondary to solitary plasmocytoma. To our knowledge, this is the first reported case of POEMS syndrome with Sjögren’s syndrome occurring in the absence of a peripheral monoclonal gammopathy, highlighting the diagnostic challenges posed by this disease and reviewing the diagnostic role of (18) F-FDG PET/CT in POEMS syndrome.