Polyneuropathy as Novel Initial Manifestation in a Case of "Nonsecretory" POEMS Syndrome with Sjögren's Syndrome. (4th January 2017)

• Record Type: Journal Article
• Authors: Liang, Minrui; Jiang, Zhixing; Lin, Zhiguang; Chen, Bobin; Zou, Hejian; Wan, Weiguo; Liu, Jun
• Other Names: Mangoni Arduino A. Academic Editor.
• Is Part Of: Case reports in medicine. Volume 2017(2017)
• Publication Date: 2017-01-04
• Subjects: Medicine -- Periodicals; Medicine -- Periodicals; Medicine; Case Reports; Periodicals; 610
• DOI: 10.1155/2017/1276759
• Languages: English
• ISSNs: 1687-9627

Abstract

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.