title: Sjögren's syndrome associated with protein losing gastroenteropathy manifested by intestinal lymphangiectasia successfully treated with prednisolone and hydroxychloroquine.
abstract: Protein-losing gastroenteropathy (PLGE), a rare manifestation of primary Sjögren's syndrome (SS), is characterized by profound edema and severe hypoalbuminemia secondary to excessive serum protein loss from the gastrointestinal tract and is clinically indistinguishable from nephrotic syndrome. We report a case of a 30-year-old Taiwanese woman with PLGE-associated SS. In addition to a positive Schirmer's test, she had eye-dryness, thirst, and high levels of anti-SSA antibodies, fulfilling SS criteria. PLGE diagnosis was highly appropriate given the clinical profile of hypoalbuminemia, hypercholesterolemia, pleural effusion, and ascites, with absent cardiac, hepatic, or renal disease. We were unable to perform technetium-99 m-labeled human serum albumin scintigraphy ((99m)Tc-HAS). However, the patient's edema and albumin level improved dramatically in response to a 3-month regime of oral prednisolone followed by oral hydroxychloroquine.
authors.affiliation: Department of Medicine, Kaohsiung Armed Forces General Hospital, Kaohsiung, Taiwan ericliao0217@gmail.com.
authors.forename: C-Y
authors.initials: CY
authors.lastname: Liao
authors.affiliation: Department of Medicine, Kaohsiung Armed Forces General Hospital, Kaohsiung, Taiwan.
authors.forename: S-T
authors.initials: ST
authors.lastname: Chien
authors.affiliation: Department of Medicine, Kaohsiung Armed Forces General Hospital, Kaohsiung, Taiwan.
authors.forename: C-C
authors.initials: CC
authors.lastname: Wang
authors.affiliation: Department of Medicine, Kaohsiung Armed Forces General Hospital, Kaohsiung, Taiwan.
authors.forename: I-H
authors.initials: IH
authors.lastname: Chen
authors.affiliation: Department of Medicine, Kaohsiung Armed Forces General Hospital, Kaohsiung, Taiwan.
authors.forename: H-W
authors.initials: HW
authors.lastname: Chiu
authors.affiliation: Department of Medicine, Kaohsiung Armed Forces General Hospital, Kaohsiung, Taiwan.
authors.forename: M-Y
authors.initials: MY
authors.lastname: Liu
authors.affiliation: Department of Medicine, Kaohsiung Armed Forces General Hospital, Kaohsiung, Taiwan.
authors.forename: C-H
authors.initials: CH
authors.lastname: Lin
authors.affiliation: Department of Medicine, Kaohsiung Armed Forces General Hospital, Kaohsiung, Taiwan.
authors.forename: R-J
authors.initials: RJ
authors.lastname: Ben
authors.affiliation: Department of Medicine, Kaohsiung Armed Forces General Hospital, Kaohsiung, Taiwan.
authors.forename: M-K
authors.initials: MK
authors.lastname: Tsai
mesh_terms: D000328:Adult; D000893:Anti-Inflammatory Agents / Q000008:administration & dosage*; D018501:Antirheumatic Agents / Q000008:administration & dosage*; D005260:Female; D006801:Humans; D006886:Hydroxychloroquine / Q000008:administration & dosage*; D008201:Lymphangiectasis, Intestinal / Q000378:metabolism* / Q000473:pathology; D011239:Prednisolone / Q000008:administration & dosage*; D011504:Protein-Losing Enteropathies / Q000188:drug therapy* / Q000378:metabolism / Q000473:pathology; D012859:Sjogren's Syndrome / Q000378:metabolism* / Q000473:pathology
chemical_list: D000893:Anti-Inflammatory Agents; D018501:Antirheumatic Agents; D006886:Hydroxychloroquine; D011239:Prednisolone
keywords: Protein-losing gastroenteropathy; Sjögren’s syndrome; intestinal lymphangiectasia