title: Clinical Presentation of Tubulointerstitial Nephritis Caused by Amyloid Light-chain Amyloidosis in a Patient with Sjögren's Syndrome.
abstract: We report a 70-year-old woman with Sjögren's syndrome who had severe renal dysfunction with mild proteinuria and elevated urinary low-molecular-weight proteins. Based on these clinical presentations, interstitial nephritis due to Sjögren's syndrome was strongly suspected. Unexpectedly, renal pathology revealed amyloid light-chain (AL) lambda-type depositions predominantly in the vasculatures with severe tubulointerstitial damage. Concentrated urine immunofixation was positive for Bence Jones lambda-type monoclonal proteins. Given the involvement in other organs, systemic AL amyloidosis was diagnosed. The patient underwent chemotherapy, but hemodialysis was ultimately instituted. It should be remembered that renal amyloidosis occurs as a clinical presentation of interstitial nephritis.
authors.affiliation: Department of Internal Medicine, Teikyo University School of Medicine, Japan.
authors.forename: Reiko
authors.initials: R
authors.lastname: Inoue
authors.forename: Yoshihide
authors.initials: Y
authors.lastname: Fujigaki
authors.forename: Kana
authors.initials: K
authors.lastname: Kobayashi
authors.forename: Yoshifuru
authors.initials: Y
authors.lastname: Tamura
authors.forename: Tatsuru
authors.initials: T
authors.lastname: Ota
authors.forename: Shigeru
authors.initials: S
authors.lastname: Shibata
authors.forename: Tsuyoshi
authors.initials: T
authors.lastname: Ishida
authors.forename: Fukuo
authors.initials: F
authors.lastname: Kondo
authors.forename: Yutaka
authors.initials: Y
authors.lastname: Yamaguchi
authors.forename: Shunya
authors.initials: S
authors.lastname: Uchida
mesh_terms: D000368:Aged; D000682:Amyloid / Q000378:metabolism; D000686:Amyloidosis / Q000150:complications* / Q000175:diagnosis; D001536:Bence Jones Protein / Q000652:urine; D001706:Biopsy; D005260:Female; D006801:Humans; D007146:Immunoglobulin lambda-Chains / Q000652:urine; D007668:Kidney / Q000473:pathology; D009395:Nephritis, Interstitial / Q000209:etiology* / Q000473:pathology; D012859:Sjogren's Syndrome / Q000150:complications*
chemical_list: D000682:Amyloid; D007146:Immunoglobulin lambda-Chains; D001536:Bence Jones Protein