Renal involvement of monoclonal immunoglobulin deposition disease associated with an unusual monoclonal immunoglobulin A glycan profile View Full Text


Ontology type: schema:ScholarlyArticle     


Article Info

DATE

2010-05-08

AUTHORS

Shuzo Kaneko, Joichi Usui, Yoshiki Narimatsu, Hiromi Ito, Hisashi Narimatsu, Masahiro Hagiwara, Shuichi Tsuruoka, Michio Nagata, Kunihiro Yamagata

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. More... »

PAGES

389-395

Identifiers

URI

http://scigraph.springernature.com/pub.10.1007/s10157-010-0285-0

DOI

http://dx.doi.org/10.1007/s10157-010-0285-0

DIMENSIONS

https://app.dimensions.ai/details/publication/pub.1009422609

PUBMED

https://www.ncbi.nlm.nih.gov/pubmed/20454825


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