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Renal involvement of monoclonal immunoglobulin deposition disease associated with an unusual monoclonal immunoglobulin A glycan profile
- Source :
- Clinical and experimental nephrology. 14(4)
- Publication Year :
- 2009
-
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.
- Subjects :
- Nephrology
Adult
Male
medicine.medical_specialty
Glycosylation
Physiology
Glomerulonephritis, Membranoproliferative
Prednisolone
Paraproteinemias
urologic and male genital diseases
Mass Spectrometry
Nephropathy
Immunoglobulin kappa-Chains
Polysaccharides
Physiology (medical)
Internal medicine
Gammopathy
medicine
Humans
Glucocorticoids
Melphalan
medicine.diagnostic_test
business.industry
Glomerulonephritis
medicine.disease
Glomerular Mesangium
Immunoglobulin A
Treatment Outcome
Mesangium
Immunology
Mesangial proliferative glomerulonephritis
Drug Therapy, Combination
Renal biopsy
business
Protein Processing, Post-Translational
Monoclonal Immunoglobulin Deposition Disease
Subjects
Details
- ISSN :
- 14377799
- Volume :
- 14
- Issue :
- 4
- Database :
- OpenAIRE
- Journal :
- Clinical and experimental nephrology
- Accession number :
- edsair.doi.dedup.....e0b8936df3b2aa95ebbd8c283c765270