Polyneuropathy as Novel Initial Manifestation in a Case of "Nonsecretory" POEMS Syndrome with Sjögren's Syndrome

Minrui Liang; Zhixing Jiang; Zhiguang Lin; Bobin Chen; Hejian Zou; Weiguo Wan; Jun Liu

doi:10.1155/2017/1276759

Polyneuropathy as Novel Initial Manifestation in a Case of "Nonsecretory" POEMS Syndrome with Sjögren's Syndrome

Case Rep Med. 2017:2017:1276759. doi: 10.1155/2017/1276759. Epub 2017 Jan 4.

Authors

Minrui Liang¹, Zhixing Jiang¹, Zhiguang Lin², Bobin Chen², Hejian Zou¹, Weiguo Wan¹, Jun Liu³

Affiliations

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

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.