title: Renal involvement of monoclonal immunoglobulin deposition disease associated with an unusual monoclonal immunoglobulin A glycan profile.
abstract: A 38-year-old man was admitted to the hospital for the evaluation of proteinuria, microscopic hematuria, and monoclonal IgA-kappa gammopathy. The initial renal pathological findings showed mesangial proliferative glomerulonephritis with endocapillary proliferation, a necrotizing lesion, and cellular crescent formation accompanied by IgA1-kappa deposition in the mesangium. Neither typical immune-complex deposits nor organized-structure deposits were detected. We diagnosed the patient with monoclonal immunoglobulin deposition disease (MIDD) associated with monoclonal IgA (mIgA). After the initiation of a monthly treatment with melphalan and predonisolone (MP therapy), the patient's serum IgA levels declined, and clinical remission was ultimately achieved. The follow-up renal biopsy showed reduced IgA-kappa staining, and both the endocapillary proliferation and the necrotizing lesion had disappeared. To elucidate the mechanism of IgA deposition, we investigated the glycan profile of the patient's serum mIgA using a mass spectrometry technique. The results revealed an unusual N-glycan profile compared to that of another patient with circulating mIgA lacking renal involvement and that of a healthy control. mIgA deposition in the mesangial area is a rare disease, and the glycan profiling of MIDD with renal involvement has not been reported previously. Thus, the present case suggests that any variation in Ig glycosylation may be a step in the pathogenesis of MIDD with renal involvement and/or contribute to some cases of IgA nephropathy.
authors.affiliation: Department of Nephrology, University of Tsukuba, Japan.
authors.forename: Syuzou
authors.initials: S
authors.lastname: Kaneko
authors.forename: Joichi
authors.initials: J
authors.lastname: Usui
authors.forename: Yoshiki
authors.initials: Y
authors.lastname: Narimatsu
authors.forename: Hiromi
authors.initials: H
authors.lastname: Ito
authors.forename: Hisashi
authors.initials: H
authors.lastname: Narimatsu
authors.forename: Masahiro
authors.initials: M
authors.lastname: Hagiwara
authors.forename: Shuichi
authors.initials: S
authors.lastname: Tsuruoka
authors.forename: Michio
authors.initials: M
authors.lastname: Nagata
authors.forename: Kunihiro
authors.initials: K
authors.lastname: Yamagata
mesh_terms: D000328:Adult; D004359:Drug Therapy, Combination; D005920:Glomerular Mesangium / Q000276:immunology* / Q000473:pathology; D015432:Glomerulonephritis, Membranoproliferative / Q000188:drug therapy / Q000276:immunology* / Q000473:pathology; D005938:Glucocorticoids / Q000627:therapeutic use; D006031:Glycosylation; D006801:Humans; D007070:Immunoglobulin A / Q000032:analysis* / Q000097:blood; D007145:Immunoglobulin kappa-Chains / Q000032:analysis* / Q000097:blood; D008297:Male; D013058:Mass Spectrometry; D008558:Melphalan / Q000627:therapeutic use; D010265:Paraproteinemias / Q000188:drug therapy / Q000276:immunology* / Q000473:pathology; D011134:Polysaccharides / Q000032:analysis* / Q000097:blood; D011239:Prednisolone / Q000627:therapeutic use; D011499:Protein Processing, Post-Translational; D016896:Treatment Outcome
chemical_list: D005938:Glucocorticoids; D007070:Immunoglobulin A; D007145:Immunoglobulin kappa-Chains; D011134:Polysaccharides; D011239:Prednisolone; D008558:Melphalan
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