Renal involvement of monoclonal immunoglobulin deposition disease associated with an unusual monoclonal immunoglobulin A glycan profile |
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Authors: | Syuzou Kaneko Joichi Usui Yoshiki Narimatsu Hiromi Ito Hisashi Narimatsu Masahiro Hagiwara Shuichi Tsuruoka Michio Nagata Kunihiro Yamagata |
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Institution: | (1) Department of Nephrology, Graduate School of Comprehensive Human Sciences, University of Tsukuba, Tsukuba 305-8575, Japan;(2) Research Center for Medical Glycoscience, National Institute of Advanced Industrial Science and Technology (AIST), 1-1-1 Umezono, Tsukuba Ibaraki, 305-8568, Japan;(3) Department of Pathology, Graduate School of Comprehensive Human Sciences, University of Tsukuba, Tsukuba Ibaraki, 305-8575, Japan; |
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Abstract: | A 38-year-old man was admitted to the hospital for the evaluation of proteinuria, microscopic hematuria, and monoclonal IgA-κ
gammopathy. The initial renal pathological findings showed mesangial proliferative glomerulonephritis with endocapillary proliferation,
a necrotizing lesion, and cellular crescent formation accompanied by IgA1-κ 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-κ 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. |
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