Protein-losing gastroenteropathy associated with primary Sjögren's syndrome: a characteristic oriental variant.
Protein-losing gastroenteropathy (PLGE) is a rare manifestation of primary Sjögren's syndrome (SS). We report a case of a 41-year-old Japanese man, who is the first male patient, with PLGE associated with primary SS. Although serum anti-SSA and SSB antibodies were detected, he had no subjective sicca symptoms. He had multiple annular erythema: a characteristic skin manifestation of Asian SS patients. A diagnosis of PLGE was made from results of (99m)Tc-labelled albumin scintigraphy and a faecal alpha-1-antitrypsin clearance test. Intravenous administration of high-dose glucocorticoid was not effective, but pulse methylprednisolone therapy alleviated disease manifestations. As all cases of PLGE associated with primary SS have been reported from East Asia, this complication could be essentially limited to Asian patients.
Nagashima
Takao
T
Division of Rheumatology and Clinical Immunology, Jichi Medical University, Yakushiji, 3311-1, Shimotsuke-shi, Tochigi, 329-0498, Japan. naga4ma@jichi.ac.jp
Hoshino
Motoaki
M
Shimoji
Shin
S
Morino
Noritsugu
N
Kamimura
Takeshi
T
Okazaki
Hitoaki
H
Minota
Seiji
S
D000328:Adult; D000418:Albumins / Q000378:metabolism; D044466:Asian People / Q000208:ethnology; D004890:Erythema / Q000208:ethnology / Q000276:immunology / Q000503:physiopathology; D041981:Gastrointestinal Tract / Q000000981:diagnostic imaging / Q000276:immunology* / Q000503:physiopathology*; D006801:Humans; D007564:Japan; D008297:Male; D008657:Metabolic Clearance Rate; D008775:Methylprednisolone / Q000627:therapeutic use; D011504:Protein-Losing Enteropathies / Q000208:ethnology / Q000276:immunology* / Q000503:physiopathology*; D044469:Racial Groups; D011877:Radionuclide Imaging; D012859:Sjogren's Syndrome / Q000150:complications* / Q000208:ethnology / Q000276:immunology; D016896:Treatment Outcome; D000515:alpha 1-Antitrypsin / Q000378:metabolism
D000418:Albumins; C043392:SERPINA1 protein, human; D000515:alpha 1-Antitrypsin; D008775:Methylprednisolone