title: Polyneuropathy as Novel Initial Manifestation in a Case of "Nonsecretory" POEMS Syndrome with Sjögren's Syndrome.
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.
authors.affiliation: Division of Rheumatology, Huashan Hospital, Shanghai, China; Institute of Rheumatology, Immunology and Allergy, Fudan University, Shanghai 200040, China.
authors.forename: Minrui
authors.initials: M
authors.lastname: Liang
authors.affiliation: Division of Rheumatology, Huashan Hospital, Shanghai, China; Institute of Rheumatology, Immunology and Allergy, Fudan University, Shanghai 200040, China.
authors.forename: Zhixing
authors.initials: Z
authors.lastname: Jiang
authors.affiliation: Department of Hematology, Huashan Hospital, Fudan University, Shanghai 200040, China.
authors.forename: Zhiguang
authors.initials: Z
authors.lastname: Lin
authors.affiliation: Department of Hematology, Huashan Hospital, Fudan University, Shanghai 200040, China.
authors.forename: Bobin
authors.initials: B
authors.lastname: Chen
authors.affiliation: Division of Rheumatology, Huashan Hospital, Shanghai, China; Institute of Rheumatology, Immunology and Allergy, Fudan University, Shanghai 200040, China.
authors.forename: Hejian
authors.initials: H
authors.lastname: Zou
authors.affiliation: Division of Rheumatology, Huashan Hospital, Shanghai, China; Institute of Rheumatology, Immunology and Allergy, Fudan University, Shanghai 200040, China.
authors.forename: Weiguo
authors.identifier: 0000-0001-6710-1330
authors.initials: W
authors.lastname: Wan
authors.affiliation: Nursing Department, Huashan Hospital, Fudan University, Shanghai 200040, China.
authors.forename: Jun
authors.identifier: 0000-0003-3875-111X
authors.initials: J
authors.lastname: Liu